Radiological features of pancreatic desmoid-type fibromatosis: A case series and systematic review

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Methods We searched PubMed, Cochrane Library, and Web of Science Core Collection and included 31 patients with pathologically proven PDFs with analyzable preoperative computed tomography (CT) and magnetic resonance imaging, including two patients from our institution and 29 patients from 28 publications. Two board-certified radiologists reviewed all images. Results The median age of the patients was 39 years, with a male dominance observed (male, 54.8% vs. female, 45.2%). Abdominal pain was the most frequent symptom, occurring in 58.1% of cases. Surgical resection was performed in all cases of PDFs, resulting in a recurrence rate of 8.3% (2/24). The tumors were most commonly located in the pancreatic tail (23/31, 74.2%). In terms of morphology, a "solid" shape was most prevalent (14/31, 45.2%), followed by a "solid and cystic" shape (9/31, 29.0%) and a "cystic" shape (8/31, 25.8%). Characteristic radiological features included heterogeneous enhancement of the solid portion of the tumors on CT scans (13/20, 65%), moderate-to-weak enhancement in the late phase on CT (16/17, 94.1%), and a presence of cystic components in the tumors (17/31, 54.8%). In 16.1% (5/31) of PDFs, the cystic component was pathologically confirmed to be a dilated pancreatic duct. Conclusion We summarized the clinical and imaging characteristics of PDF. Although the incidence may not be high, cystic components suggesting a dilated pancreatic duct within the tumor are unique imaging features in PDF. desmoid pancreatic tumor systematic review computed tomography magnetic resonance imaging Figures Figure 1 Figure 2 Figure 3 Key points 1. We have comprehensively summarized the demographic, clinical, and radiological features of PDF. 2. Cystic components within the tumors, suggestive of a dilated pancreatic duct, are unique imaging features of PDF. Introduction Desmoid tumor, also known as desmoid-type fibromatosis or aggressive fibromatosis, is a locally aggressive but non-metastasizing deep-seated (myo)fibroblastic neoplasm with infiltrative growth and propensity for local recurrence [1]. Desmoid-type fibromatosis are estimated to affect 2–4 patients per 1 million population per year [2]. Patients tend to be young, with a median age of 37–39 years [2-4]. Most desmoid-type fibromatosis occur sporadically; however, they may also occur in association with the hereditary syndrome such as familial adenomatous polyposis (FAP) [5]. In a previous study, tumor locations were as follows: extremity (42%), abdominal or retroperitoneal cavity (20%), abdominal wall (17%), and chest wall (15%) [3]. Desmoid-type fibromatosis have rarely been reported to involve the pancreas, with 42 cases reported in the literature, typically as single cases [6]. Moreover, surgical resection is performed for 85.7% of pancreatic desmoid-type fibromatosis (PDF), with a recurrence rate of 4.8% [6], which is lower than that for desmoid-type fibromatosis (44.3%) [3]. While the majority of literature concerning PDF has focused on its pathological, histological, and clinical findings, knowledge of the radiological imaging findings of this tumor has been limited. To the best of our knowledge, this is the most comprehensive systematic review to date on the radiological features of PDF. The purpose of this study was to summarize the demographic and clinical data of patients and the imaging findings of PDF to identify features that are helpful in diagnosis. Material and methods Study selection We searched PubMed, the Cochrane Library, and the Web of Science Core Collection using the following search terms on September 3, 2023, without any language or date limits: ("fibromatosis, aggressive"[mh] OR "fibroma"[mh] OR desmoid*[tiab] OR fibromatosis[tiab] OR fibroma[tiab]) AND (pancreas[mh] OR "pancreatic diseases"[mh] OR pancreas[tiab] OR pancreatic[tiab]) AND (“diagnostic imaging"[sh] OR diagnostic imaging[mh] OR radiologic*[tiab] OR radiology[tiab] OR imaging[tiab] OR "magnetic resonance"[tiab] OR "computed tomography"[tiab] OR "computerized tomography"[tiab] OR MRI[tiab] OR CT[tiab]) for PubMed; ([mh "fibromatosis, aggressive"] OR [mh "fibroma"] OR (desmoid* OR fibromatosis OR fibroma):ti,ab,kw) and ([mh pancreas] OR [mh "pancreatic diseases"] OR (pancreas OR pancreatic):ti,ab,kw) for Cochrane Library; (fibroma OR desmoid* OR fibromatosis OR fibromatoses) AND (pancreas OR pancreatic) to the Web of Science Core Collection. Publications were considered eligible if they included all of the following criteria: The inclusion criteria were as follows: The content was relevant to PDF; Either a magnetic resonance imaging (MRI) or computed tomography (CT) image of the tumor was available; The exclusion criteria were as follows: Duplicate cases; The full text was unavailable. We obtained institutional review board exemption for including two unpublished cases with pathologically proven PDF and their preoperative CT or MRI findings obtained at our hospital. Data were acquired in compliance with all applicable regulations of the Health Insurance Portability and Accountability Act. This study was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) 2020 statement [7]. Data analyses Two board-certified radiologists independently reviewed all studies and the CT and MR images of the eligible examinations. When discrepancies arose between the two reviewers, another board-certified radiologist made a tie-breaker decision. Collected data The following data were collected: Demographics Patient age Sex Clinical Presenting complaint Recurrence after treatment (presence or absence) Survival outcomes (survive or dead) Follow-up duration (month since diagnosis) Radiological Tumor size and location Tumor margin status (well- or ill-defined) Tumor morphology (solid, cystic, or solid and cystic) CT attenuation (high density: higher than muscles; intermediate density: between cerebrospinal fluid [CSF] and muscles; low density: lower than CSF) CT enhancement of the solid portion (homogeneous or heterogeneous; strong: stronger than the pancreas; moderate: almost the same as the pancreas; weak: weaker than the pancreas). T2-weighted image (T2WI) signal intensity (high intensity, higher than fat; intermediate intensity, between fat and muscles; low intensity, lower than muscles) Fat-suppressed (fs) T2WI signal intensity (high intensity: higher than CSF; intermediate intensity: between CSF and muscle; low intensity: lower than muscle) T1-weighted image (T1WI) and fat-suppressed T1WI signal intensities (high intensity: higher than muscles; intermediate intensity: between muscles and CSF; low intensity: lower than CSF) The presence or absence of hemorrhage MRI enhancement of the solid portion (homogeneous or heterogeneous; strong, stronger than the pancreas; moderate, almost the same as the pancreas; weak, weaker than the pancreas) The presence or absence of diffusion restriction The maximum standardized uptake value on F-18 fluorodeoxyglucose uptake on positron emission tomography (PET)/CT Quality assessment For a systematic review of case-based studies, we used a tool proposed by Murad et al. to evaluate the methodological quality of case reports/series [8]. This tool is based on the eight signaling questions in four domains (selection, ascertainment, causality, and reporting) and has been widely used in previous studies [9-12]. Results Study selection A total of 208 abstracts were screened according to the PRISMA 2020 guidelines [7], of which 39 potentially eligible studies were assessed for review. After excluding 11 studies, 28 studies with 29 cases met the criteria for the systematic review [6, 13-39]. The identification process used in these studies is summarized in Fig. 1. The publication year of the included studies ranged from 1996 to 2023. We added two unpublished cases from our hospital (Table 1), resulting in a final study cohort of 31 patients who underwent PDF. Risk of bias assessment As we extracted data from case-based studies, where the selection method was rarely mentioned, a selection bias may have been introduced. Tumor size was available for 30 of the 31 cases (96.7%). Treatment strategies and outcomes were ascertained for 27 of 31 (87.1%) and 23 of 31 (74.2%) patients, respectively. The follow-up duration of the patients ranged from 2 months to 5 years. Because we evaluated cases using preoperative CT or MR, our research can be replicated by other investigators. Demographic and clinical data The demographic and clinical data of the 31 patients are summarized in Table 2. The median age at diagnosis was 39 years (range, 13–75 years), with no evidence of differences in the incidence by age from 10- to 70-year-olds. More male patients (17/31, 54.8%) were affected than were female patients (14/31, 45.2%). A total of 5.0% (1/20) and 33.3% (7/21) of the patients had a family history of FAP and a history of abdominal surgery, respectively. The most frequent chief complaint was abdominal pain (18/31, 58.1%), followed by absence of symptoms (7/31, 22.6%). Surgery alone (26/27, 96.3%) was the most commonly used treatment strategy, and the incidence of tumor recurrence after complete resection was low (2/24, 8.3%). In 16.1% (5/31) of the PDFs, the cystic component was pathologically confirmed to be a dilated pancreatic duct. Moreover, 24 cases of PDFs showed nuclear staining for β-catenin. Other immunohistochemical results for the PDFs are summarized in Table 2. Radiological findings Radiological findings are summarized in Table 3. The median tumor size was 65.5 mm (range: 19–284 mm). The most frequent tumor site was the tail of the pancreas (23/31, 74.2%), followed by the head (4/31,12.9%) and body (4/31,12.9%). Solid shape (14/31, 45.2%) was the most frequent morphology, followed by solid and cystic shapes (9/31, 29.0%) and cystic shape (8/31, 25.8%). The tumor margins were ill-defined (17/31, 54.8%) more often than were well-defined (14/31, 45.2%). The solid portion was most likely to show intermediate density on CT (5/5, 100%), weak enhancement in the arterial phase on CT (9/9, 100%), moderate-to-weak enhancement in the late phase on CT (16/17, 94.1%), intermediate-to-high intensity on T2WI/fsT2WI (4/4, 100%), intermediate-to-high intensity on T1WI (3/3, 100%) /fat-suppressed T1WI (2/2, 100%) and non-restricted diffusion (1/2, 50%). The enhancement patterns of the solid portion on MRI varied among patients. The cystic portion showed intermediate-to-low density on CT (5/5, 100%), high intensity on T2WI/fat-suppressed T2WI (2/2, 100%), intermediate-to-high intensity on T1WI (3/3, 100%), and high intensity on fat-suppressed T1WI (1/1, 100%). Hemorrhage was observed in a limited number of patients (1/31, 3.2%). 18F-FDG-PET/CT images were available in only two cases, but avid FDG uptake was observed in both cases. We observed no apparent differences in the imaging findings between FAP-associated and non-FAP-associated PDF. Histological and immunohistochemical findings of our two cases Surgical resection was performed in two cases characterized by a cystic component on imaging examinations (Figs. 2 and 3). Histological sections from these two cases confirmed that the cystic component, observed in both tumors on imaging studies, corresponded to a dilated pancreatic duct. In these cases, tumors infiltrating the pancreatic parenchyma around the dilated ducts were noted. For both, the pancreatic ductal epithelium exhibited reactive changes, with no evidence of dysplasia, and the presence of pancreatic ductal obstruction or pancreatitis remained unclear. Immunohistochemical studies revealed that the tumor cells tested positive for beta-catenin and negative for CD34, CD117, desmin, and S100. Discussion In this study, we summarized the demographic, clinical, and radiological features of 29 patients with PDFs from 28 publications and two additional patients from our hospital. The median age of the patients was 39 years, with a male predominance (male, 54.8% vs. female, 45.2%) and abdominal pain being the most frequent symptom (58.1%). Surgical resection was performed in all PDFs, with a recurrence rate of 8.3% (2/24 patients). Tumors were most frequently observed in the pancreatic tail (23/31, 74.2%). Characteristic radiological features included heterogeneous enhancement of the solid portion of the tumors on CT (13/20, 65%), moderate-to-weak enhancement in the late phase (16/17, 94.1%), and the frequent presence of cystic components in tumors (17/31, 54.8%). To the best of our knowledge, this is the largest systematic review of radiological findings of PDFs. Desmoid-type fibromatosis are rare neoplasms that occur sporadically or in the context of FAP. According to a study by Nieuwenhuis et al. [5], at least 7.5% of desmoid-type fibromatosis are associated with FAP. The calculated incidence of sporadic desmoid-type fibromatosis was 3.42 per million person-years, whereas, for the FAP population, the incidence was 2,784 per million person-years, indicating an over 800-fold increased risk of desmoid-type fibromatosis in patients with FAP. However, owing to the low incidence of FAP, most desmoid-type fibromatosis encountered in daily clinical practice are sporadic, as only 5 % (1/20) of the patients had concomitant FAP in the present study. Surgical resection remains the cornerstone of treatment for desmoid-type fibromatosis when R0 and function-sparing resection is achievable [40, 41]. However, in certain cases, achieving a resection with wide, clear margins to prevent tumor recurrence poses challenges due to the tumor's locally invasive nature. In recent years, options such as observation and the use of anti-inflammatory drugs have emerged as potential treatment choices for the management of desmoid-type fibromatosis [41]. In the case of PDFs, there exists a reported case in which tumor control was achieved through partial cystectomy and administration of a cyclooxygenase-2 inhibitor [36]; however, there are no comprehensive reports demonstrating the efficacy of non-steroidal anti-inflammatory drugs alone. Additional clinical trials and molecular analyses should be performed to test the effects of non-steroidal anti-inflammatory drugs in PDFs. Recurrence rates of desmoid-type fibromatosis have been reported to be 8% in cases associated with FAP and 12% in sporadic cases [5], and our study found that the recurrence rate of PDFs (8.3%) is generally consistent with that of previous studies. Immunohistochemistry for specific cell markers of various origins is an effective tool for distinguishing PDF from other pancreatic tumors. PDF exhibits distinctive immunohistochemical features, primarily characterized by nuclear staining of β-catenin, which sets it apart from other mesenchymal tumors of pancreatic origin [42]. In this study, nuclear β-catenin staining was observed in 24 cases of PDFs. Other immunohistochemical results were also generally consistent with those of previous research [43]. Expression was negative for CD34, CD117, S-100, and desmin, which excluded a gastrointestinal stromal tumor, schwannoma, leiomyoma, and leiomyosarcoma, respectively [44]. A comprehensive understanding of the radiological findings of PDF is lacking owing to the rarity of this condition. We found that the "solid" morphology was the most common, followed by the "solid and cystic" and "cystic" morphology. Interestingly, 29.4% (5/17) of the PDFs contained characteristic cystic components on imaging, and the cystic components were pathologically identified as pancreatic duct dilatation. We believe that the cystic component within the tumor, consisting of dilated pancreatic ducts, is a unique imaging and pathological finding of PDFs that is not observed in desmoid-type fibromatosis at other sites. Regarding the mechanism of pancreatic ductal dilatation, some reports suggest that it may be a secondary change associated with pancreatic ductal obstruction [6, 38]; however, in these two previously reported cases, pancreatic duct obstruction due to the tumor was not confirmed pathologically. In contrast, in our two cases, the presence of pancreatic ductal obstruction or pancreatitis was unclear based on imaging and pathological findings. Clarke-Brodber et al. also reported that none of the 42 patients who underwent PDFs showed clinical features of pancreatitis [6], suggesting that the mechanism of pancreatic ductal obstruction is unlikely. Therefore, we speculated that the primary cause of pancreatic ductal dilatation in PDF is the disruption of the supporting tissue of the pancreatic ducts due to tumor infiltration rather than mechanical ductal obstruction by the tumors. Differential diagnosis includes pancreatic tumors such as neuroendocrine neoplasms, solid pseudopapillary neoplasms, serous cystic neoplasms, and mucinous cystic neoplasms [6]. The continuity of cystic components within the tumor during PDF may be a strong differentiating feature that is not observed in other pancreatic tumors. This study had some limitations. First, the number of patients was small, although this report included the largest cohort of patients who underwent PDFs with analyzable radiological findings. Second, the images were not acquired using a standardized protocol. Third, information regarding prognosis, post-treatment imaging changes, apparent diffusion coefficients, and standardized uptake values was limited. Future studies using this information and the sequences are required to further clarify the characteristics of PDF. In conclusion, we have comprehensively summarized the demographic, clinical, and radiological features of PDF. PDF occurs most frequently in the pancreatic tail, and the patterns of imaging findings are classified into "solid," "cystic," and "solid and cystic" shapes, with solid shapes being the most common. We also found that cystic components within the tumors, suggestive of a dilated pancreatic duct, are unique imaging features of PDF. The recognition of these factors may facilitate the preoperative diagnosis of PDF. Abbreviations CSF, cerebrospinal fluid FAP, familial adenomatous polyposis PDF, pancreatic desmoid-type fibromatosis T1WI, T1-weighted imaging T2WI, T2-weighted imaging Declarations Author Contribution Guarantors of integrity of entire study, M.K; study concepts/study design or data acquisition or data analysis/interpretation, all authors; manuscript drafting or manuscript revision for important intellectual content, all authors; approval of final version of submitted manuscript, all authors; literature research, T.T. and R.K.; clinical studies, T.T. and R.K.; statistical analysis, T.T. and R.K.; and manuscript editing, all authors. Acknowledgement We thank Editage (www.editage.jp) for English language editing. We also express our gratitude to Dr. Nobuyoshi Hiraoka, Department of Diagnostic Pathology, National Cancer Center Hospital, for providing expert pathological advice. 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Demographic, clinical, and radiological data of two patients with pancreatic desmoid-type fibromatosis in our hospital Demographic & Clinical data Patients 1 2 Age (years) 20 22 Sex Female Female Abdominal pain Yes Yes Family history of FAP No No History of abdominal trauma or surgery No No Surgery Yes Yes Chemotherapy No No Recurrence No No Current status Survive Survive Follow up duration (month) 18 174 Radiological data Tumor size (mm) 284 54 Tumor site Tail Tail Tumor margin Well-defined Ill-defined Morphology Solid and cystic Solid and cystic Solid portion CT attenuation Intermediate Intermediate CT enhancement Homogeneous Homogeneous In the arterial phase Weak Weak In the late phase Weak Moderate T2WI Intermediate Intermediate Fat-suppressed T2WI High T1WI High Intermediate Fat-suppressed T1WI High MRI enhancement Homogeneous Homogeneous In the arterial phase Weak In the late phase Strong Strong Diffusion restriction No Cystic portion CT attenuation Low Low T2WI High High Fat-suppressed T2WI High High T1WI Intermediate Low Fat-suppressed T1WI High Hemorrhage Yes No Metastasis No No Gray cell: not available, FAP: familial adenomatous polyposis, CT: computed tomography, T2WI: T2-weighted imaging, T1WI: T1-weighted imaging, MRI: magnetic resonance imaging Table 2. Demographic and clinical information of the 31 patients with pancreatic desmoid-type fibromatosis Demographic Median age at diagnosis (years [range]) (n = 31) 39 [13–75] Sex Male = 17, Female = 14 Family history of FAP 1/20 (5.0%) History of abdominal trauma 0/9 (0%) History of abdominal surgery 7/21 (33.3%) Clinical Abdominal pain 18/31 (58.1%) No symptoms 7/31 (22.6%) Nausea 6/31 (19.4%) Vomiting 6/31 (19.4%) Abdominal bloating 5/31 (16.1%) Anorexia 3/31 (9.7%) Weight loss 3/31 (9.7%) Back pain 3/31 (9.7%) Fever 2/31 (6.5%) Chest pain 1/31 (3.2%) Diarrhea 1/31 (3.2%) Constipation 1/31 (3.2%) Melena 1/31 (3.2%) Dysphasia 1/31 (3.2%) Initial treatment strategy (n = 27) Surgery alone 26/27 (96.3%) Surgery and COX-2 inhibitor 1/27 (3.7%) Recurrence after surgery 2/24 (8.3%) Patient status Survive = 24/25 (96.0%), Death = 1/25 (4.0%) Follow up duration (median [range]) (n = 24) 19 months [2–174] Pathological Pancreatic duct dilatation 5/31 (16.1%) IHC Positive Negative Nuclear β-catenin (n = 24) 24 0 α-SMA (n = 16) 12 4 CD34 (n = 15) 2 13 CD117 (n = 14) 1 13 Desmin (n = 13) 1 12 S100 (n = 13) 1 12 Vimentin (n = 5) 5 0 ALK (n = 5) 0 5 Ki67 (n = 4) 0 4 DOG1 (n = 4) 0 4 Pancytokeratin (n = 4) 0 4 Calretinin (n = 3) 0 3 EMA (n = 2) 0 2 Caldesmon (n = 2) 0 2 MUC4 (n = 2) 0 2 WT1 (n = 2) 0 2 ER (n = 2) 0 2 STAT6 (n = 2) 0 2 PgR (n = 1) 0 1 CD10 (n = 1) 1 0 Bc12 (n = 1) 0 1 Cytokeratin (n = 1) 1 0 D2-40 (n = 1) 0 1 MC (n = 1) 0 1 MDM2 (n = 1) 0 1 CDK4 (n = 1) 0 1 CK5/6 (n = 1) 0 1 Actin (n = 1) 1 0 Sox10 (n = 1) 0 1 FAP = familial adenomatous polyposis, COX-2 = cyclooxygenase-2, IHC = immunohistochemistry Table 3. Radiological characteristics of the 31 patients with pancreatic desmoid-type fibromatosis Parameters Size (median [range]) (n = 30) 65.5 mm [19–284] Location Head 4/31 (12.9%) Body 4/31 (12.9%) Tail 23/31 (74.2%) Morphology Solid 14/31 (45.2%) Cystic 8/31 (25.8%) Solid and cystic 9/31 (29.0%) Tumor margin Well-defined 14/31 (45.2%) Ill-defined 17/31 (54.8%) CT attenuation of solid portion Intermediate 5/5 (100%) CT attenuation of cystic portion Intermediate 1/5 (20%) Low 4/5 (80%) CT enhancement of solid portion Homogeneous 7/20 (35%) Heterogeneous 13/20 (65%) In the arterial phase Weak 9/9 (100%) In the late phase Strong 1/17 (5.9%) Moderate 8/17 (47.1%) Weak 8/17 (47.1%) T2WI signal intensity of solid portion High 2/4 (50%) Intermediate 2/4 (50%) Fat-suppressed T2WI signal intensity of solid portion High 1/4 (25%) Intermediate 3/4 (75%) T2WI signal intensity of cystic portion High 2/2 (100%) Fat-suppressed T2WI signal intensity of cystic portion High 2/2 (100%) T1WI signal intensity of solid portion High 1/3 (33.3%) Intermediate 2/3 (66.7%) Fat-suppressed T1WI signal intensity of solid portion High 1/2 (50%) Intermediate 1/2 (50%) T1WI signal intensity of cystic portion Intermediate 1/2 (50%) Low 1/2 (50%) Fat-suppressed T1WI signal intensity of cystic portion High 1/1 (100%) Hemorrhage 1/31 (3.2%) MRI enhancement of solid portion Homogeneous 2/3 (66.7%) Heterogeneous 1/3 (33.3%) In the arterial phase Weak 1/1 (100%) In the late phase Strong 2/3 (66.7%) Weak 1/3 (33.3%) Diffusion restriction 1/2 (50%) FDG uptake on PET/CT Avid 2/2 (100%) No metastasis 7/7 (100%) CT: computed tomography, T2WI: T2-weighted imaging, T1WI: T1-weighted imaging, MRI: magnetic resonance imaging, FDG: F-18 fluorodeoxyglucose, PET: positron emission tomography Additional Declarations No competing interests reported. Cite Share Download PDF Status: Published Journal Publication published 15 Sep, 2024 Read the published version in Abdominal Radiology → Version 1 posted Editorial decision: Revision requested 06 Aug, 2024 Reviews received at journal 06 Aug, 2024 Reviews received at journal 02 Aug, 2024 Reviewers agreed at journal 26 Jul, 2024 Reviewers agreed at journal 24 Jul, 2024 Reviewers invited by journal 24 Jul, 2024 Editor assigned by journal 18 Jul, 2024 Submission checks completed at journal 18 Jul, 2024 First submitted to journal 18 Jul, 2024 You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. We do this by developing innovative software and high quality services for the global research community. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-4765339","acceptedTermsAndConditions":true,"allowDirectSubmit":false,"archivedVersions":[],"articleType":"Research Article","associatedPublications":[],"authors":[{"id":336681814,"identity":"b85e34da-5cb6-4109-b574-af394415ae0b","order_by":0,"name":"Tomoya Tanishima","email":"","orcid":"","institution":"National Cancer Centre Japan","correspondingAuthor":false,"prefix":"","firstName":"Tomoya","middleName":"","lastName":"Tanishima","suffix":""},{"id":336681816,"identity":"cf51a05d-ddde-4186-95e5-031896707813","order_by":1,"name":"Ryo Kurokawa","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAAxklEQVRIiWNgGAWjYBACCQhlY8cPohIKiNeSlizZANJiQLyWQ4wbDoBoYrRIzsh9+Lii4gCz8fnViR8eGDDI84sdwK9FWiLd2PDMmTt8ZjfebpYAOsxw5uwE/FrkJNLYJBvbnjGb3Ti7AaQlweA2cVoOM26ecXbzD6K0SMO0bODv3UacLZI9z5gNG86kJUvc4N1mkWAgQdgvEsfTGB82VACjsv/s5ps/Kmzk+aUJaEHSDFYpQaxyEOA/QIrqUTAKRsEoGEkAAGJDQe5BrfibAAAAAElFTkSuQmCC","orcid":"","institution":"The University of Tokyo","correspondingAuthor":true,"prefix":"","firstName":"Ryo","middleName":"","lastName":"Kurokawa","suffix":""},{"id":336681818,"identity":"eb12a4d5-4f9f-4076-a894-258cbe7d45d0","order_by":2,"name":"Miyuki Sone","email":"","orcid":"","institution":"National Cancer Centre Japan","correspondingAuthor":false,"prefix":"","firstName":"Miyuki","middleName":"","lastName":"Sone","suffix":""},{"id":336681820,"identity":"701af1d9-a94f-4da9-a8b4-47bce58f5ef0","order_by":3,"name":"Yudai Nakai","email":"","orcid":"","institution":"The University of Tokyo","correspondingAuthor":false,"prefix":"","firstName":"Yudai","middleName":"","lastName":"Nakai","suffix":""},{"id":336681822,"identity":"db2f5aa5-d9ca-48d6-ad05-baa734373eef","order_by":4,"name":"Osamu Abe","email":"","orcid":"","institution":"The University of Tokyo","correspondingAuthor":false,"prefix":"","firstName":"Osamu","middleName":"","lastName":"Abe","suffix":""},{"id":336681823,"identity":"4cf06cdd-6470-496f-b496-f4a64a112f86","order_by":5,"name":"Masahiko Kusumoto","email":"","orcid":"","institution":"National Cancer Centre Japan","correspondingAuthor":false,"prefix":"","firstName":"Masahiko","middleName":"","lastName":"Kusumoto","suffix":""}],"badges":[],"createdAt":"2024-07-19 00:08:18","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-4765339/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-4765339/v1","draftVersion":[],"editorialEvents":[{"content":"https://doi.org/10.1007/s00261-024-04570-8","type":"published","date":"2024-09-15T15:58:00+00:00"}],"editorialNote":"","failedWorkflow":false,"files":[{"id":62660583,"identity":"99c76248-19ef-4e24-bd4b-635fbaea8f8d","added_by":"auto","created_at":"2024-08-17 02:34:42","extension":"jpg","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":252607,"visible":true,"origin":"","legend":"\u003cp\u003eFlow diagram of study identification\u003c/p\u003e","description":"","filename":"Figure1.jpg","url":"https://assets-eu.researchsquare.com/files/rs-4765339/v1/269e751e1dd950a035de5ebb.jpg"},{"id":62660582,"identity":"f1402e83-b42e-4b9e-9fc5-c80bafff3005","added_by":"auto","created_at":"2024-08-17 02:34:42","extension":"jpg","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":737247,"visible":true,"origin":"","legend":"\u003cp\u003ePancreatic desmoid-type fibromatosis in a 22-year-old female presenting with abdominal bloating, constipation, and abdominal pain (case 1). CT shows a 27-cm well-delineated tumor with a solid and cystic morphology (A, plain; B, arterial-phase; C, delayed-phase). The solid portion shows delayed enhancement on contrast-enhanced CT (A, B, C) and MRI (H), high intensity on fat-suppressed T1WI (D) and coronal T1WI (E), intermediate intensity on coronal T2WI (F), high intensity on fat-suppressed coronal T2WI (G), and homogeneous enhancement (H). The cystic portion shows high intensity on coronal T2WI (F) and fat-suppressed coronal T2WI (G), and high intensity on fat-suppressed T1WI (D) and coronal T1WI (E). Most of the cystic portions are continuous (white arrows), some with hemorrhage (white arrowheads). Diffusion restriction is not observed with the mean ADC value of the solid portion of 1.50 × 10\u003csup\u003e-3\u003c/sup\u003emm\u003csup\u003e2\u003c/sup\u003e/s (I, J). Low-power magnification with hematoxylin and eosin stain shows desmoid-type fibromatosis with prominent pancreatic ductal dilation (K, white arrow). High-power magnification with hematoxylin and eosin stain shows pancreatic ductal epithelium showing reactive changes without evidence of dysplasia (L). Corresponding β-catenin nuclear positivity is observed (M). Abbreviations: CT, computed tomography; MRI, magnetic resonance imaging; T1WI, T1-weighted imaging; T2WI, T2-weighted imaging; ADC, apparent diffusion coefficient\u003c/p\u003e","description":"","filename":"Figure2.jpg","url":"https://assets-eu.researchsquare.com/files/rs-4765339/v1/e49d1e6792e36a954cccc07c.jpg"},{"id":62661653,"identity":"bd7173cd-a512-4e1f-89d9-3178b5003450","added_by":"auto","created_at":"2024-08-17 02:42:42","extension":"jpg","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":963557,"visible":true,"origin":"","legend":"\u003cp\u003ePancreatic desmoid-type fibromatosis in a 20-year-old asymptomatic female (case 2). CT shows a 4-cm well-delineated tumor with a solid and cystic morphology (A, plain; B, arterial-phase; C, delayed-phase). The solid portion shows delayed enhancement on contrast-enhanced CT (A, B, C) and MRI (G), intermediate intensity on coronal T2 True Fast Imaging with Steady State Precession (True FISP) (D) and T1WI (F), and homogeneous enhancement (G). The cystic portion shows high intensity on coronal True FISP (D) and MRCP (E), and low intensity on T1WI (F). Most of the cystic portions are continuous (A–E, white arrows). Low-power magnification with hematoxylin and eosin stain shows desmoid-type fibromatosis with prominent pancreatic ductal dilation (H, white arrows). High-power magnification with hematoxylin and eosin stain shows pancreatic ductal epithelium showing reactive changes without evidence of dysplasia (I). Corresponding β-catenin nuclear positivity is observed (not shown). Abbreviations: CT, computed tomography; MRI, magnetic resonance imaging; T2WI, T2-weighted imaging; T1WI, T1-weighted imaging; MRCP, magnetic resonance cholangiopancreatography\u003c/p\u003e","description":"","filename":"Figure3.jpg","url":"https://assets-eu.researchsquare.com/files/rs-4765339/v1/7255986523b1b529d0a8e10a.jpg"},{"id":64619209,"identity":"42df9bfd-0ba6-4c88-9406-193ab1ac3a51","added_by":"auto","created_at":"2024-09-16 16:12:44","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":2634426,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-4765339/v1/af6edd92-68db-4917-9a16-21f82b423eba.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Radiological features of pancreatic desmoid-type fibromatosis: A case series and systematic review","fulltext":[{"header":"Key points","content":"\u003cp\u003e1.\u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp;We have comprehensively summarized the demographic, clinical, and radiological features of PDF.\u003c/p\u003e\n\u003cp\u003e2. \u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp; Cystic components within the tumors, suggestive of a dilated pancreatic duct, are unique imaging features of PDF.\u003c/p\u003e"},{"header":"Introduction","content":"\u003cp\u003eDesmoid tumor, also known as\u0026nbsp;desmoid-type fibromatosis or aggressive fibromatosis, is a locally aggressive but non-metastasizing deep-seated (myo)fibroblastic neoplasm with infiltrative growth and propensity for local recurrence\u0026nbsp;[1].\u0026nbsp;Desmoid-type fibromatosis are\u0026nbsp;estimated to affect\u0026nbsp;2\u0026ndash;4\u0026nbsp;patients per 1 million population per year\u0026nbsp;[2]. Patients tend to be young, with a median age of 37\u0026ndash;39 years\u0026nbsp;[2-4].\u0026nbsp;Most desmoid-type fibromatosis occur sporadically; however, they may also occur in association with the hereditary syndrome such as familial adenomatous polyposis (FAP)\u0026nbsp;[5]. In a previous study, tumor locations were as follows: extremity (42%), abdominal or retroperitoneal cavity (20%), abdominal wall (17%), and chest wall (15%)\u0026nbsp;[3]. Desmoid-type fibromatosis have rarely been reported to involve the pancreas, with 42 cases reported in the literature, typically as single cases\u0026nbsp;[6]. Moreover, surgical resection is performed for 85.7% of pancreatic desmoid-type fibromatosis (PDF), with a recurrence rate of 4.8%\u0026nbsp;[6], which is lower than that for desmoid-type fibromatosis (44.3%)\u0026nbsp;[3].\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eWhile the majority of literature concerning PDF has focused on its pathological, histological, and clinical findings, knowledge of the radiological imaging findings of this tumor has been limited. To the best of our knowledge, this is the most comprehensive systematic review to date on the radiological features of PDF. The purpose of this study was to summarize the demographic and clinical data of patients and the imaging findings of PDF to identify features that are helpful in diagnosis.\u003c/p\u003e"},{"header":"Material and methods","content":"\u003cp\u003e\u003cstrong\u003e\u003cem\u003eStudy selection\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eWe searched PubMed, the Cochrane Library, and the Web of Science Core Collection using the following search terms on September 3, 2023, without any language or date limits:\u003c/p\u003e\n\u003cul\u003e\n \u003cli\u003e(\"fibromatosis, aggressive\"[mh] OR \"fibroma\"[mh] OR desmoid*[tiab] OR fibromatosis[tiab] OR fibroma[tiab]) AND (pancreas[mh] OR \"pancreatic diseases\"[mh] OR pancreas[tiab] OR pancreatic[tiab]) AND (“diagnostic imaging\"[sh] OR diagnostic imaging[mh] OR radiologic*[tiab] OR radiology[tiab] OR imaging[tiab] OR \"magnetic resonance\"[tiab] OR \"computed tomography\"[tiab] OR \"computerized tomography\"[tiab] OR MRI[tiab] OR CT[tiab]) for PubMed;\u003c/li\u003e\n \u003cli\u003e([mh \"fibromatosis, aggressive\"] OR [mh \"fibroma\"] OR (desmoid* OR fibromatosis OR fibroma):ti,ab,kw) and ([mh pancreas] OR [mh \"pancreatic diseases\"] OR (pancreas OR pancreatic):ti,ab,kw) for Cochrane Library;\u003c/li\u003e\n \u003cli\u003e(fibroma OR desmoid* OR fibromatosis OR fibromatoses) AND (pancreas OR pancreatic)\u0026nbsp;to the Web of Science Core Collection.\u003c/li\u003e\n\u003c/ul\u003e\n\u003cp\u003ePublications were considered eligible if they included all of the following criteria:\u003c/p\u003e\n\u003cp\u003eThe inclusion criteria were as follows:\u003c/p\u003e\n\u003cul\u003e\n \u003cli\u003eThe content was relevant to PDF;\u003c/li\u003e\n \u003cli\u003eEither a magnetic resonance imaging (MRI) or computed tomography (CT) image of the tumor was available;\u003c/li\u003e\n\u003c/ul\u003e\n\u003cp\u003eThe exclusion criteria were as follows:\u003c/p\u003e\n\u003cul\u003e\n \u003cli\u003eDuplicate cases;\u003c/li\u003e\n \u003cli\u003eThe full text was unavailable.\u003c/li\u003e\n\u003c/ul\u003e\n\u003cp\u003eWe obtained institutional review board exemption for including two unpublished cases with pathologically proven PDF and their preoperative CT or MRI findings obtained at our hospital. Data were acquired in compliance with all applicable regulations of the Health Insurance Portability and Accountability Act.\u003c/p\u003e\n\u003cp\u003e\u0026nbsp;This study was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) 2020 statement\u0026nbsp;[7].\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cem\u003eData analyses\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eTwo board-certified radiologists independently reviewed all studies and the CT and MR images of the eligible examinations. When discrepancies arose between the two reviewers, another board-certified radiologist made a tie-breaker decision.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cem\u003eCollected data\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe following data were collected:\u003c/p\u003e\n\u003cp\u003eDemographics\u003c/p\u003e\n\u003cul\u003e\n \u003cli\u003ePatient age\u003c/li\u003e\n \u003cli\u003eSex\u003c/li\u003e\n\u003c/ul\u003e\n\u003cp\u003eClinical\u003c/p\u003e\n\u003cul\u003e\n \u003cli\u003ePresenting complaint\u003c/li\u003e\n \u003cli\u003eRecurrence after treatment (presence or absence)\u003c/li\u003e\n \u003cli\u003eSurvival outcomes (survive or dead)\u003c/li\u003e\n \u003cli\u003eFollow-up duration (month since diagnosis)\u003c/li\u003e\n\u003c/ul\u003e\n\u003cp\u003eRadiological\u003c/p\u003e\n\u003cul\u003e\n \u003cli\u003eTumor size and location\u003c/li\u003e\n \u003cli\u003eTumor margin status (well- or ill-defined)\u003c/li\u003e\n \u003cli\u003eTumor morphology (solid, cystic, or solid and cystic)\u003c/li\u003e\n \u003cli\u003eCT attenuation (high density: higher than muscles; intermediate density: between cerebrospinal fluid [CSF] and muscles; low density: lower than CSF)\u003c/li\u003e\n \u003cli\u003eCT enhancement of the solid portion (homogeneous or heterogeneous; strong: stronger than the pancreas; moderate: almost the same as the pancreas; weak: weaker than the pancreas).\u003c/li\u003e\n \u003cli\u003eT2-weighted image (T2WI) signal intensity (high intensity, higher than fat; intermediate intensity, between fat and muscles; low intensity, lower than\u0026nbsp;muscles)\u003c/li\u003e\n \u003cli\u003eFat-suppressed (fs) T2WI signal intensity (high intensity: higher than CSF; intermediate intensity: between CSF and muscle; low intensity: lower than muscle)\u003c/li\u003e\n \u003cli\u003eT1-weighted image (T1WI) and fat-suppressed T1WI signal intensities (high intensity: higher than muscles; intermediate intensity: between muscles and CSF; low intensity: lower than CSF)\u003c/li\u003e\n \u003cli\u003eThe presence or absence of hemorrhage\u003c/li\u003e\n \u003cli\u003eMRI enhancement of the solid portion (homogeneous or heterogeneous; strong, stronger than the pancreas; moderate, almost the same as the pancreas; weak, weaker than the pancreas)\u003c/li\u003e\n \u003cli\u003eThe presence or absence of diffusion restriction\u003c/li\u003e\n \u003cli\u003eThe maximum standardized uptake value on F-18 fluorodeoxyglucose uptake on positron emission tomography (PET)/CT\u003c/li\u003e\n\u003c/ul\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cem\u003eQuality assessment\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eFor a systematic review of case-based studies, we used a tool proposed by Murad et al. to evaluate the methodological quality of case reports/series [8]. This tool is based on the eight signaling questions in four domains (selection, ascertainment, causality, and reporting) and has been widely used in previous studies [9-12].\u003c/p\u003e"},{"header":"Results","content":"\u003cp\u003e\u003cstrong\u003e\u003cem\u003eStudy selection\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eA total of 208 abstracts were screened according to the PRISMA 2020 guidelines\u0026nbsp;[7], of which 39 potentially eligible studies were assessed for review. After excluding 11 studies, 28 studies with 29 cases met the criteria for the systematic review\u0026nbsp;[6, 13-39]. The identification process used in these studies is summarized in Fig. 1. The publication year of the included studies ranged from 1996 to 2023. We added two unpublished cases from our hospital (Table 1), resulting in a final study cohort of 31 patients who underwent PDF.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cem\u003eRisk of bias assessment\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eAs we extracted data from case-based studies, where the selection method was rarely mentioned, a selection bias may have been introduced. Tumor size was available for 30 of the 31 cases (96.7%). Treatment strategies and outcomes were ascertained for 27 of 31 (87.1%) and 23 of 31 (74.2%) patients, respectively. The follow-up duration of the patients ranged from 2 months to 5 years. Because we evaluated cases using preoperative CT or MR, our research can be replicated by other investigators.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cem\u003eDemographic and clinical data\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe demographic and clinical data of the 31 patients are summarized in Table 2. The median age at diagnosis was 39 years (range, 13–75 years), with no evidence of differences in the incidence by age from 10- to 70-year-olds. More male patients (17/31, 54.8%) were affected than were female patients (14/31, 45.2%). A total of 5.0% (1/20) and 33.3% (7/21) of the patients had a family history of FAP and a history of abdominal surgery, respectively.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eThe most frequent chief complaint was abdominal pain (18/31, 58.1%), followed by absence of symptoms (7/31, 22.6%). Surgery alone (26/27, 96.3%) was the most commonly used treatment strategy, and the incidence of tumor recurrence after complete resection was low (2/24, 8.3%). In 16.1% (5/31) of the PDFs, the cystic component was pathologically confirmed to be a dilated pancreatic duct. Moreover, 24 cases of PDFs showed nuclear staining for β-catenin. Other immunohistochemical results for the PDFs are summarized in Table 2.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cem\u003eRadiological findings\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eRadiological findings are summarized in Table 3. The median tumor size was 65.5 mm (range: 19–284 mm). The most frequent tumor site was the tail of the pancreas (23/31, 74.2%), followed by the head (4/31,12.9%) and body (4/31,12.9%). Solid shape (14/31, 45.2%) was the most frequent morphology, followed by solid and cystic shapes (9/31, 29.0%) and cystic shape (8/31, 25.8%). The tumor margins were ill-defined (17/31, 54.8%) more often than were well-defined (14/31, 45.2%). The solid portion was most likely to show intermediate density on CT (5/5, 100%), weak enhancement in the arterial phase on CT (9/9, 100%), moderate-to-weak enhancement in the late phase on CT (16/17, 94.1%), intermediate-to-high intensity on T2WI/fsT2WI (4/4, 100%), intermediate-to-high intensity on T1WI (3/3, 100%) /fat-suppressed T1WI (2/2, 100%) and non-restricted diffusion (1/2, 50%). The enhancement patterns of the solid portion on MRI varied among patients. The cystic portion showed intermediate-to-low density on CT (5/5, 100%), high intensity on T2WI/fat-suppressed T2WI (2/2, 100%), intermediate-to-high intensity on T1WI (3/3, 100%), and high intensity on fat-suppressed T1WI (1/1, 100%).\u0026nbsp;Hemorrhage was observed in a limited number of patients (1/31, 3.2%).\u0026nbsp;18F-FDG-PET/CT images were available in only two cases, but avid FDG uptake was observed in both cases. We observed no apparent differences in the imaging findings between FAP-associated and non-FAP-associated PDF.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cem\u003eHistological and immunohistochemical findings of our two cases\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eSurgical resection was performed in two cases characterized by a cystic component on imaging examinations (Figs. 2 and 3). Histological sections from these two cases confirmed that the cystic component, observed in both tumors on imaging studies, corresponded to a dilated pancreatic duct. In these cases, tumors infiltrating the pancreatic parenchyma around the dilated ducts were noted. For both, the pancreatic ductal epithelium exhibited reactive changes, with no evidence of dysplasia, and the presence of pancreatic ductal obstruction or pancreatitis remained unclear. Immunohistochemical studies revealed that the tumor cells tested positive for beta-catenin and negative for CD34, CD117, desmin, and S100.\u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003eIn this study, we summarized the demographic, clinical, and radiological features of 29 patients with PDFs from 28 publications and two additional patients from our hospital. The median age of the patients was 39 years, with a male predominance (male, 54.8% vs. female, 45.2%) and abdominal pain being the most frequent symptom (58.1%). Surgical resection was performed in all PDFs, with a recurrence rate of 8.3% (2/24 patients). Tumors were most frequently observed in the pancreatic tail (23/31, 74.2%). Characteristic radiological features included heterogeneous enhancement of the solid portion of the tumors on CT (13/20, 65%), moderate-to-weak enhancement in the late phase (16/17, 94.1%), and the frequent presence of cystic components in tumors (17/31, 54.8%). To the best of our knowledge, this is the largest systematic review of radiological findings of PDFs.\u003c/p\u003e\n\u003cp\u003eDesmoid-type fibromatosis are rare neoplasms that occur sporadically or in the context of FAP. According to a study by Nieuwenhuis et al.\u0026nbsp;[5], at least 7.5% of desmoid-type fibromatosis are associated with FAP. The calculated incidence of sporadic desmoid-type fibromatosis was 3.42 per million person-years, whereas, for the FAP population, the incidence was 2,784 per million person-years, indicating an over 800-fold increased risk of desmoid-type fibromatosis in patients with FAP. However, owing to the low incidence of FAP, most desmoid-type fibromatosis encountered in daily clinical practice are sporadic, as only 5 % (1/20) of the patients had concomitant FAP in the present study.\u003c/p\u003e\n\u003cp\u003eSurgical resection remains the cornerstone of treatment for desmoid-type fibromatosis when R0 and function-sparing resection is achievable\u0026nbsp;[40, 41]. However, in certain cases, achieving a resection with wide, clear margins to prevent tumor recurrence poses challenges due to the tumor\u0026apos;s locally invasive nature. In recent years, options such as observation and the use of anti-inflammatory drugs have emerged as potential treatment choices for the management of desmoid-type fibromatosis\u0026nbsp;[41]. In the case of PDFs, there exists a reported case in which tumor control was achieved through partial cystectomy and administration of a cyclooxygenase-2 inhibitor\u0026nbsp;[36]; however, there are no comprehensive reports demonstrating the efficacy of\u0026nbsp;non-steroidal anti-inflammatory drugs\u0026nbsp;alone. Additional clinical trials and molecular analyses should be performed to test the effects of\u0026nbsp;non-steroidal anti-inflammatory drugs\u0026nbsp;in PDFs. Recurrence rates of desmoid-type fibromatosis have been reported to be 8% in cases associated with FAP and 12% in sporadic cases\u0026nbsp;[5], and our study found that the recurrence rate of PDFs (8.3%) is generally consistent with that of previous studies.\u003c/p\u003e\n\u003cp\u003eImmunohistochemistry for specific cell markers of various origins is an effective tool for distinguishing PDF from other pancreatic tumors.\u0026nbsp;PDF exhibits distinctive immunohistochemical features, primarily characterized by nuclear staining of \u0026beta;-catenin, which sets it apart from other mesenchymal tumors of pancreatic origin\u0026nbsp;[42]. In this study, nuclear \u0026beta;-catenin staining was observed in 24 cases of PDFs. Other immunohistochemical results were also generally consistent with those of previous research\u0026nbsp;[43]. Expression was negative for CD34, CD117, S-100, and desmin, which excluded a gastrointestinal stromal tumor, schwannoma, leiomyoma, and leiomyosarcoma, respectively\u0026nbsp;[44].\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eA comprehensive understanding of the radiological findings of PDF is lacking owing to the rarity of this condition. We found that the \u0026quot;solid\u0026quot; morphology was the most common, followed by the \u0026quot;solid and cystic\u0026quot; and \u0026quot;cystic\u0026quot; morphology. Interestingly, 29.4% (5/17) of the PDFs contained characteristic cystic components on imaging, and the cystic components were pathologically identified as pancreatic duct dilatation. We believe that the cystic component within the tumor, consisting of dilated pancreatic ducts, is a unique imaging and pathological finding of PDFs that is not observed in desmoid-type fibromatosis at other sites. Regarding the mechanism of pancreatic ductal dilatation, some reports suggest that it may be a secondary change associated with pancreatic ductal obstruction\u0026nbsp;[6, 38]; however, in these two previously reported cases, pancreatic duct obstruction due to the tumor was not confirmed pathologically. In contrast, in our two cases, the presence of pancreatic ductal obstruction or pancreatitis was unclear based on imaging and pathological findings. Clarke-Brodber et al. also reported that none of the 42 patients who underwent PDFs showed clinical features of pancreatitis\u0026nbsp;[6], suggesting that the mechanism of pancreatic ductal obstruction is unlikely. Therefore, we speculated that the primary cause of pancreatic ductal dilatation in PDF is the disruption of the supporting tissue of the pancreatic ducts due to tumor infiltration rather than mechanical ductal obstruction by the tumors. Differential diagnosis includes pancreatic tumors such as neuroendocrine neoplasms, solid pseudopapillary neoplasms, serous cystic neoplasms, and mucinous cystic neoplasms\u0026nbsp;[6]. The continuity of cystic components within the tumor during PDF may be a strong differentiating feature that is not observed in other pancreatic tumors.\u003c/p\u003e\n\u003cp\u003e\u0026nbsp;This study had some limitations. First, the number of patients was small, although this report included the largest cohort of patients who underwent PDFs with analyzable radiological findings. Second, the images were not acquired using a standardized protocol. Third, information regarding prognosis, post-treatment imaging changes, apparent diffusion coefficients, and standardized uptake values was limited. Future studies using this information and the sequences are required to further clarify the characteristics of PDF.\u003c/p\u003e\n\u003cp\u003eIn conclusion, we have comprehensively summarized the demographic, clinical, and radiological features of PDF. PDF occurs most frequently in the pancreatic tail, and the patterns of imaging findings are classified into \u0026quot;solid,\u0026quot; \u0026quot;cystic,\u0026quot; and \u0026quot;solid and cystic\u0026quot; shapes, with solid shapes being the most common. We also found that cystic components within the tumors, suggestive of a dilated pancreatic duct, are unique imaging features of PDF. The recognition of these factors may facilitate the preoperative diagnosis of PDF.\u003c/p\u003e"},{"header":"Abbreviations","content":"\u003cp\u003eCSF, cerebrospinal fluid\u003c/p\u003e\n\u003cp\u003eFAP,\u0026nbsp;familial adenomatous polyposis\u003c/p\u003e\n\u003cp\u003ePDF, pancreatic desmoid-type fibromatosis\u003c/p\u003e\n\u003cp\u003eT1WI, T1-weighted imaging\u003c/p\u003e\n\u003cp\u003eT2WI, T2-weighted imaging\u003c/p\u003e"},{"header":"Declarations","content":"\u003ch2\u003eAuthor Contribution\u003c/h2\u003e\u003cp\u003eGuarantors of integrity of entire study, M.K; study concepts/study design or data acquisition or data analysis/interpretation, all authors; manuscript drafting or manuscript revision for important intellectual content, all authors; approval of final version of submitted manuscript, all authors; literature research, T.T. and R.K.; clinical studies, T.T. and R.K.; statistical analysis, T.T. and R.K.; and manuscript editing, all authors.\u003c/p\u003e\u003ch2\u003eAcknowledgement\u003c/h2\u003e\u003cp\u003eWe thank Editage (www.editage.jp) for English language editing. We also express our gratitude to Dr. Nobuyoshi Hiraoka, Department of Diagnostic Pathology, National Cancer Center Hospital, for providing expert pathological advice. Additionally, we acknowledge Ms. Keiko Kato, a librarian at National Cancer Center, for her technical assistance in literature searches.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n\u003cli\u003eFritchie KJ, Crago AM, Van de Rijn M (2020) Soft Tissue and Bone Tumours, 5th. World Health Organization, pp 93-95\u003c/li\u003e\n\u003cli\u003eReitamo JJ, Hayry P, Nykyri E, Saxen E (1982) The desmoid tumor. I. Incidence, sex-, age- and anatomical distribution in the Finnish population. Am J Clin Pathol, 77(6):665-673\u003c/li\u003e\n\u003cli\u003eCrago AM, Denton B, Salas S, et al. (2013) A prognostic nomogram for prediction of recurrence in desmoid fibromatosis. Ann Surg, 258(2):347-353\u003c/li\u003e\n\u003cli\u003eSalas S, Dufresne A, Bui B, et al. 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Pediatr Neonatol, 64(3):344-346\u003c/li\u003e\n\u003cli\u003eDallaire DFB, Dallaire DE, Perigny DM (2018) Pancreatic desmoid tumor: A rare case with radiologic-pathologic correlation. Radiol Case Rep, 13(5):1079-1083\u003c/li\u003e\n\u003cli\u003eGerleman R, Mortensen MB, Detlefsen S (2015) Desmoid Tumor of the Pancreas: Case Report and Review of a Rare Entity. Int J Surg Pathol, 23(7):579-584\u003c/li\u003e\n\u003cli\u003eHagiwara K, Mihara K, Aiura K, Shito M (2020) Successful outcomes after laparoscopic spleen-preserving pancreatic resection for a desmoid tumor: A case report. Int J Surg Case Rep, 74:117-119\u003c/li\u003e\n\u003cli\u003eJafri SF, Obaisi O, Vergara GG, et al. (2017) Desmoid type fibromatosis: A case report with an unusual etiology. World J Gastrointest Oncol, 9(9):385-389\u003c/li\u003e\n\u003cli\u003eJia C, Tian B, Dai C, Wang X, Bu X, Xu F (2014) Idiopathic desmoid-type fibromatosis of the pancreatic head: case report and literature review. 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Am J Case Rep, 23:e937324\u003c/li\u003e\n\u003cli\u003eMeyer A, Szajnbok P, Koszka AJM, Pezzutti D, Segatelli V, Monteiro J, Jr. (2021) A rare sporadic pancreatic desmoid fibromatosis with splenic vein invasion diagnosed by CT scan-guided core needle biopsy: a case report with possible differential diagnosis from metastatic colorectal or renal cancer. J Surg Case Rep, 2021(6):rjab257\u003c/li\u003e\n\u003cli\u003eMourra N, Ghorra C, Arrive L (2015) An Unusual Solid and Cystic Pancreatic Tumor in a 20-Year-Old Woman. Desmoid Tumor: Fibromatosis. Gastroenterology, 149(3):e5-6\u003c/li\u003e\n\u003cli\u003eNursal TZ, Abbasoglu O (2003) Sporadic hereditary pancreatic desmoid tumor: a new entity? J Clin Gastroenterol, 37(2):186-188\u003c/li\u003e\n\u003cli\u003ePark CG, Lee YN, Kim WY (2021) Desmoid type fibromatosis of the distal pancreas: A case report. 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J Med Case Rep, 9:104\u003c/li\u003e\n\u003cli\u003eTorres JC, Xin C (2018) An unusual finding in a desmoid-type fibromatosis of the pancreas: a case report and review of the literature. J Med Case Rep, 12(1):123\u003c/li\u003e\n\u003cli\u003eTsukamoto Y, Imakita M, Nishitani A, Ito T, Izukura M, Hirota S (2016) Pancreatic desmoid-type fibromatosis with beta-catenin gene mutation-Report of a case and review of the literature. Pathol Res Pract, 212(5):484-489\u003c/li\u003e\n\u003cli\u003eWang YC, Wong JU (2016) Complete remission of pancreatic head desmoid tumor treated by COX-2 inhibitor-a case report. World J Surg Oncol, 14(1):190\u003c/li\u003e\n\u003cli\u003eWeiss ES, Burkart AL, Yeo CJ (2006) Fibromatosis of the remnant pancreas after pylorus-preserving pancreaticoduodenectomy. J Gastrointest Surg, 10(5):679-688\u003c/li\u003e\n\u003cli\u003eXu B, Zhu LH, Wu JG, Wang XF, Matro E, Ni JJ (2013) Pancreatic solid cystic desmoid tumor: case report and literature review. World J Gastroenterol, 19(46):8793-8798\u003c/li\u003e\n\u003cli\u003eXuesong D (2020) Pancreatic desmoid tumor with unusual imaging features. Pancreatology, 20(5):1015-1016\u003c/li\u003e\n\u003cli\u003eHuang K, Wang CM, Chen JG, et al. (2014) Prognostic factors influencing event-free survival and treatments in desmoid-type fibromatosis: analysis from a large institution. Am J Surg, 207(6):847-854\u003c/li\u003e\n\u003cli\u003eZeng WG, Zhou ZX, Liang JW, et al. (2014) Prognostic factors for desmoid tumor: a surgical series of 233 patients at a single institution. Tumour Biol, 35(8):7513-7521\u003c/li\u003e\n\u003cli\u003eBhattacharya B, Dilworth HP, Iacobuzio-Donahue C, et al. (2005) Nuclear beta-catenin expression distinguishes deep fibromatosis from other benign and malignant fibroblastic and myofibroblastic lesions. Am J Surg Pathol, 29(5):653-659\u003c/li\u003e\n\u003cli\u003eAskan G, Basturk O (2022) Mesenchymal Tumors Involving the Pancreas: A Clinicopathologic Analysis and Review of the Literature. Turk Patoloji Derg, 38(1):46-53\u003c/li\u003e\n\u003cli\u003eMiettinen M, Lasota J (2006) Gastrointestinal stromal tumors: review on morphology, molecular pathology, prognosis, and differential diagnosis. Arch Pathol Lab Med, 130(10):1466-1478\u003c/li\u003e\n\u003c/ol\u003e"},{"header":"Tables","content":"\u003cp\u003e\u003cstrong\u003eTable 1. Demographic, clinical, and radiological data of two patients with pancreatic desmoid-type fibromatosis in our hospital\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n\u003ctable border=\"1\" cellspacing=\"0\" cellpadding=\"0\" width=\"567\"\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd width=\"19.047619047619047%\" rowspan=\"11\"\u003e\n \u003cp\u003eDemographic\u003c/p\u003e\n \u003cp\u003e\u0026amp;\u003c/p\u003e\n \u003cp\u003eClinical data\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"41.7989417989418%\" colspan=\"2\" height=\"39\"\u003e\n \u003cp\u003ePatients\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"19.576719576719576%\" height=\"39\"\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"19.576719576719576%\" height=\"39\"\u003e\n \u003cp\u003e2\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"51.63398692810458%\" colspan=\"2\" height=\"39\"\u003e\n \u003cp\u003eAge (years)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.18300653594771%\" height=\"39\"\u003e\n \u003cp\u003e20\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.18300653594771%\" height=\"39\"\u003e\n \u003cp\u003e22\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"51.63398692810458%\" colspan=\"2\" height=\"39\"\u003e\n \u003cp\u003eSex\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.18300653594771%\" height=\"39\"\u003e\n \u003cp\u003eFemale\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.18300653594771%\" height=\"39\"\u003e\n \u003cp\u003eFemale\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"51.63398692810458%\" colspan=\"2\" height=\"39\"\u003e\n \u003cp\u003eAbdominal pain\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.18300653594771%\" height=\"39\"\u003e\n \u003cp\u003eYes\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.18300653594771%\" height=\"39\"\u003e\n \u003cp\u003eYes\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"51.63398692810458%\" colspan=\"2\" height=\"39\"\u003e\n \u003cp\u003eFamily history of FAP\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.18300653594771%\" height=\"39\"\u003e\n \u003cp\u003eNo\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.18300653594771%\" height=\"39\"\u003e\n \u003cp\u003eNo\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"51.63398692810458%\" colspan=\"2\" height=\"39\"\u003e\n \u003cp\u003eHistory of abdominal trauma or surgery\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.18300653594771%\" height=\"39\"\u003e\n \u003cp\u003eNo\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.18300653594771%\" height=\"39\"\u003e\n \u003cp\u003eNo\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"51.63398692810458%\" colspan=\"2\" height=\"39\"\u003e\n \u003cp\u003eSurgery\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.18300653594771%\" height=\"39\"\u003e\n \u003cp\u003eYes\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.18300653594771%\" height=\"39\"\u003e\n \u003cp\u003eYes\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"51.63398692810458%\" colspan=\"2\" height=\"39\"\u003e\n \u003cp\u003eChemotherapy\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.18300653594771%\" height=\"39\"\u003e\n \u003cp\u003eNo\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.18300653594771%\" height=\"39\"\u003e\n \u003cp\u003eNo\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"51.63398692810458%\" colspan=\"2\" height=\"39\"\u003e\n \u003cp\u003eRecurrence\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.18300653594771%\" height=\"39\"\u003e\n \u003cp\u003eNo\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.18300653594771%\" height=\"39\"\u003e\n \u003cp\u003eNo\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"51.63398692810458%\" colspan=\"2\" height=\"39\"\u003e\n \u003cp\u003eCurrent status\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.18300653594771%\" height=\"39\"\u003e\n \u003cp\u003eSurvive\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.18300653594771%\" height=\"39\"\u003e\n \u003cp\u003eSurvive\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"51.63398692810458%\" colspan=\"2\" height=\"39\"\u003e\n \u003cp\u003eFollow up duration (month)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.18300653594771%\" height=\"39\"\u003e\n \u003cp\u003e18\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.18300653594771%\" height=\"39\"\u003e\n \u003cp\u003e174\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"19.047619047619047%\" rowspan=\"23\"\u003e\n \u003cp\u003eRadiological data\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"41.7989417989418%\" colspan=\"2\" height=\"39\"\u003e\n \u003cp\u003eTumor size (mm)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"19.576719576719576%\" height=\"39\"\u003e\n \u003cp\u003e284\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"19.576719576719576%\" height=\"39\"\u003e\n \u003cp\u003e54\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"51.63398692810458%\" colspan=\"2\" height=\"39\"\u003e\n \u003cp\u003eTumor site\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.18300653594771%\" height=\"39\"\u003e\n \u003cp\u003eTail\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.18300653594771%\" height=\"39\"\u003e\n \u003cp\u003eTail\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"51.63398692810458%\" colspan=\"2\" height=\"39\"\u003e\n \u003cp\u003eTumor margin\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.18300653594771%\" height=\"39\"\u003e\n \u003cp\u003eWell-defined\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.18300653594771%\" height=\"39\"\u003e\n \u003cp\u003eIll-defined\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"51.63398692810458%\" colspan=\"2\" height=\"39\"\u003e\n \u003cp\u003eMorphology\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.18300653594771%\" height=\"39\"\u003e\n \u003cp\u003eSolid and cystic\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.18300653594771%\" height=\"39\"\u003e\n \u003cp\u003eSolid and cystic\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"21.350762527233115%\" rowspan=\"12\"\u003e\n \u003cp\u003eSolid portion\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"30.28322440087146%\" height=\"39\"\u003e\n \u003cp\u003eCT attenuation\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.18300653594771%\" height=\"39\"\u003e\n \u003cp\u003eIntermediate\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.18300653594771%\" height=\"39\"\u003e\n \u003cp\u003eIntermediate\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"38.50415512465374%\" height=\"39\"\u003e\n \u003cp\u003eCT enhancement\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"30.74792243767313%\" height=\"39\"\u003e\n \u003cp\u003eHomogeneous\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"30.74792243767313%\" height=\"39\"\u003e\n \u003cp\u003eHomogeneous\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"38.50415512465374%\" height=\"39\"\u003e\n \u003cp\u003eIn the arterial phase\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"30.74792243767313%\" height=\"39\"\u003e\n \u003cp\u003eWeak\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"30.74792243767313%\" height=\"39\"\u003e\n \u003cp\u003eWeak\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"38.50415512465374%\" height=\"39\"\u003e\n \u003cp\u003eIn the late phase\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"30.74792243767313%\" height=\"39\"\u003e\n \u003cp\u003eWeak\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"30.74792243767313%\" height=\"39\"\u003e\n \u003cp\u003eModerate\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"38.50415512465374%\" height=\"39\"\u003e\n \u003cp\u003eT2WI\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"30.74792243767313%\" height=\"39\"\u003e\n \u003cp\u003eIntermediate\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"30.74792243767313%\" height=\"39\"\u003e\n \u003cp\u003eIntermediate\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"38.50415512465374%\" height=\"53\"\u003e\n \u003cp\u003eFat-suppressed\u003c/p\u003e\n \u003cp\u003eT2WI\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"30.74792243767313%\" height=\"53\"\u003e\n \u003cp\u003eHigh\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"30.74792243767313%\" height=\"53\" bgcolor=\"#F2F2F2\"\u003e\u003cbr\u003e\u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"38.50415512465374%\" height=\"39\"\u003e\n \u003cp\u003eT1WI\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"30.74792243767313%\" height=\"39\"\u003e\n \u003cp\u003eHigh\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"30.74792243767313%\" height=\"39\"\u003e\n \u003cp\u003eIntermediate\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"38.50415512465374%\" height=\"53\"\u003e\n \u003cp\u003eFat-suppressed\u003c/p\u003e\n \u003cp\u003eT1WI\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"30.74792243767313%\" height=\"53\"\u003e\n \u003cp\u003eHigh\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"30.74792243767313%\" height=\"53\" bgcolor=\"#F2F2F2\"\u003e\u003cbr\u003e\u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"38.50415512465374%\" height=\"39\"\u003e\n \u003cp\u003eMRI enhancement\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"30.74792243767313%\" height=\"39\"\u003e\n \u003cp\u003eHomogeneous\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"30.74792243767313%\" height=\"39\"\u003e\n \u003cp\u003eHomogeneous\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"38.50415512465374%\" height=\"39\"\u003e\n \u003cp\u003eIn the arterial phase\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"30.74792243767313%\" height=\"39\" bgcolor=\"#F2F2F2\"\u003e\u003cbr\u003e\u003c/td\u003e\n \u003ctd width=\"30.74792243767313%\" height=\"39\"\u003e\n \u003cp\u003eWeak\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"38.50415512465374%\" height=\"39\"\u003e\n \u003cp\u003eIn the late phase\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"30.74792243767313%\" height=\"39\"\u003e\n \u003cp\u003eStrong\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"30.74792243767313%\" height=\"39\"\u003e\n \u003cp\u003eStrong\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"38.50415512465374%\" height=\"39\"\u003e\n \u003cp\u003eDiffusion restriction\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"30.74792243767313%\" height=\"39\"\u003e\n \u003cp\u003eNo\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"30.74792243767313%\" height=\"39\" bgcolor=\"#F2F2F2\"\u003e\u003cbr\u003e\u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"21.350762527233115%\" rowspan=\"5\"\u003e\n \u003cp\u003eCystic portion\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"30.28322440087146%\" height=\"39\"\u003e\n \u003cp\u003eCT attenuation\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.18300653594771%\" height=\"39\"\u003e\n \u003cp\u003eLow\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.18300653594771%\" height=\"39\"\u003e\n \u003cp\u003eLow\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"38.50415512465374%\" height=\"39\"\u003e\n \u003cp\u003eT2WI\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"30.74792243767313%\" height=\"39\"\u003e\n \u003cp\u003eHigh\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"30.74792243767313%\" height=\"39\"\u003e\n \u003cp\u003eHigh\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"38.50415512465374%\" height=\"53\"\u003e\n \u003cp\u003eFat-suppressed\u003c/p\u003e\n \u003cp\u003eT2WI\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"30.74792243767313%\" height=\"53\"\u003e\n \u003cp\u003eHigh\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"30.74792243767313%\" height=\"53\"\u003e\n \u003cp\u003eHigh\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"38.50415512465374%\" height=\"39\"\u003e\n \u003cp\u003eT1WI\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"30.74792243767313%\" height=\"39\"\u003e\n \u003cp\u003eIntermediate\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"30.74792243767313%\" height=\"39\"\u003e\n \u003cp\u003eLow\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"38.50415512465374%\" height=\"53\"\u003e\n \u003cp\u003eFat-suppressed\u003c/p\u003e\n \u003cp\u003eT1WI\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"30.74792243767313%\" height=\"53\"\u003e\n \u003cp\u003eHigh\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"30.74792243767313%\" height=\"53\"\u003e\u003cbr\u003e\u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"51.63398692810458%\" colspan=\"2\" height=\"39\"\u003e\n \u003cp\u003eHemorrhage\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.18300653594771%\" height=\"39\"\u003e\n \u003cp\u003eYes\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.18300653594771%\" height=\"39\"\u003e\n \u003cp\u003eNo\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"51.63398692810458%\" colspan=\"2\" height=\"39\"\u003e\n \u003cp\u003eMetastasis\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.18300653594771%\" height=\"39\"\u003e\n \u003cp\u003eNo\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.18300653594771%\" height=\"39\"\u003e\n \u003cp\u003eNo\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n\u003c/table\u003e\n\u003cp\u003eGray cell: not available, FAP: familial adenomatous polyposis, CT: computed tomography, T2WI: T2-weighted imaging, T1WI: T1-weighted imaging, MRI: magnetic resonance imaging\u003c/p\u003e\n\u003cp\u003e\u003cbr\u003e\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eTable 2. Demographic and clinical information of the 31 patients with pancreatic desmoid-type fibromatosis\u003c/strong\u003e\u003c/p\u003e\n\u003ctable border=\"1\" cellspacing=\"0\" cellpadding=\"0\" width=\"567\"\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\" bgcolor=\"#F2F2F2\"\u003e\n \u003cp\u003eDemographic\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"49.029982363315696%\" colspan=\"2\" height=\"24\" bgcolor=\"#F2F2F2\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003eMedian age at diagnosis (years [range]) (n = 31)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"49.029982363315696%\" colspan=\"2\" height=\"24\"\u003e\n \u003cp\u003e39 [13\u0026ndash;75]\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"23\"\u003e\n \u003cp\u003eSex\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"49.029982363315696%\" colspan=\"2\" height=\"23\"\u003e\n \u003cp\u003eMale = 17, Female = 14\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003eFamily history of FAP\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"49.029982363315696%\" colspan=\"2\" height=\"24\"\u003e\n \u003cp\u003e1/20 (5.0%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003eHistory of abdominal trauma\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"49.029982363315696%\" colspan=\"2\" height=\"24\"\u003e\n \u003cp\u003e0/9 (0%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003eHistory of abdominal surgery\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"49.029982363315696%\" colspan=\"2\" height=\"24\"\u003e\n \u003cp\u003e7/21 (33.3%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\" bgcolor=\"#F2F2F2\"\u003e\n \u003cp\u003eClinical\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"49.029982363315696%\" colspan=\"2\" height=\"24\" bgcolor=\"#F2F2F2\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"23\"\u003e\n \u003cp\u003eAbdominal pain\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"49.029982363315696%\" colspan=\"2\" height=\"23\"\u003e\n \u003cp\u003e18/31 (58.1%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003eNo symptoms\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"49.029982363315696%\" colspan=\"2\" height=\"24\"\u003e\n \u003cp\u003e7/31 (22.6%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003eNausea\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"49.029982363315696%\" colspan=\"2\" height=\"24\"\u003e\n \u003cp\u003e6/31 (19.4%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003eVomiting\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"49.029982363315696%\" colspan=\"2\" height=\"24\"\u003e\n \u003cp\u003e6/31 (19.4%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003eAbdominal bloating\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"49.029982363315696%\" colspan=\"2\" height=\"24\"\u003e\n \u003cp\u003e5/31 (16.1%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"23\"\u003e\n \u003cp\u003eAnorexia\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"49.029982363315696%\" colspan=\"2\" height=\"23\"\u003e\n \u003cp\u003e3/31 (9.7%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003eWeight loss\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"49.029982363315696%\" colspan=\"2\" height=\"24\"\u003e\n \u003cp\u003e3/31 (9.7%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003eBack pain\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"49.029982363315696%\" colspan=\"2\" height=\"24\"\u003e\n \u003cp\u003e3/31 (9.7%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003eFever\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"49.029982363315696%\" colspan=\"2\" height=\"24\"\u003e\n \u003cp\u003e2/31 (6.5%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003eChest pain\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"49.029982363315696%\" colspan=\"2\" height=\"24\"\u003e\n \u003cp\u003e1/31 (3.2%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"23\"\u003e\n \u003cp\u003eDiarrhea\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"49.029982363315696%\" colspan=\"2\" height=\"23\"\u003e\n \u003cp\u003e1/31 (3.2%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003eConstipation\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"49.029982363315696%\" colspan=\"2\" height=\"24\"\u003e\n \u003cp\u003e1/31 (3.2%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003eMelena\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"49.029982363315696%\" colspan=\"2\" height=\"24\"\u003e\n \u003cp\u003e1/31 (3.2%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003eDysphasia\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"49.029982363315696%\" colspan=\"2\" height=\"24\"\u003e\n \u003cp\u003e1/31 (3.2%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003eInitial treatment strategy (n = 27)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"49.029982363315696%\" colspan=\"2\" height=\"24\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"23\"\u003e\n \u003cp\u003eSurgery alone\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"49.029982363315696%\" colspan=\"2\" height=\"23\"\u003e\n \u003cp\u003e26/27 (96.3%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003eSurgery and COX-2 inhibitor\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"49.029982363315696%\" colspan=\"2\" height=\"24\"\u003e\n \u003cp\u003e1/27 (3.7%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003eRecurrence after surgery\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"49.029982363315696%\" colspan=\"2\" height=\"24\"\u003e\n \u003cp\u003e2/24 (8.3%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003ePatient status\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"49.029982363315696%\" colspan=\"2\" height=\"24\"\u003e\n \u003cp\u003eSurvive = 24/25 (96.0%), Death = 1/25 (4.0%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003eFollow up duration (median [range]) (n = 24)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"49.029982363315696%\" colspan=\"2\" height=\"24\"\u003e\n \u003cp\u003e19 months [2\u0026ndash;174]\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"23\" bgcolor=\"#F2F2F2\"\u003e\n \u003cp\u003ePathological\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"49.029982363315696%\" colspan=\"2\" height=\"23\" bgcolor=\"#F2F2F2\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\" bgcolor=\"white\"\u003e\n \u003cp\u003ePancreatic duct dilatation\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"49.029982363315696%\" colspan=\"2\" height=\"24\" bgcolor=\"white\"\u003e\n \u003cp\u003e5/31 (16.1%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003eIHC\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003ePositive\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003eNegative\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003eNuclear \u0026beta;-catenin (n = 24)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e24\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003e\u0026alpha;-SMA (n = 16)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e12\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e4\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"23\"\u003e\n \u003cp\u003eCD34 (n = 15)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"23\"\u003e\n \u003cp\u003e2\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"23\"\u003e\n \u003cp\u003e13\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003eCD117 (n = 14)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e13\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003eDesmin (n = 13)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e12\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003eS100 (n = 13)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e12\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003eVimentin (n = 5)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e5\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"23\"\u003e\n \u003cp\u003eALK (n = 5)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"23\"\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"23\"\u003e\n \u003cp\u003e5\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"25\"\u003e\n \u003cp\u003eKi67\u0026nbsp;(n = 4)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"25\"\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"25\"\u003e\n \u003cp\u003e4\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003eDOG1 (n = 4)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e4\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003ePancytokeratin (n = 4)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e4\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003eCalretinin (n = 3)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e3\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"23\"\u003e\n \u003cp\u003eEMA (n = 2)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"23\"\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"23\"\u003e\n \u003cp\u003e2\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003eCaldesmon (n = 2)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e2\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003eMUC4 (n = 2)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e2\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003eWT1 (n = 2)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e2\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003eER (n = 2)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e2\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"23\"\u003e\n \u003cp\u003eSTAT6 (n = 2)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"23\"\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"23\"\u003e\n \u003cp\u003e2\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003ePgR (n = 1)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003eCD10 (n = 1)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003eBc12 (n = 1)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003eCytokeratin (n = 1)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"23\"\u003e\n \u003cp\u003eD2-40 (n = 1)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"23\"\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"23\"\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003eMC (n = 1)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003eMDM2 (n = 1)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003eCDK4 (n = 1)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003eCK5/6 (n = 1)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"23\"\u003e\n \u003cp\u003eActin (n = 1)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"23\"\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"23\"\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"50.970017636684304%\" height=\"24\"\u003e\n \u003cp\u003eSox10 (n = 1)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"24.514991181657848%\" height=\"24\"\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n\u003c/table\u003e\n\u003cp\u003eFAP = familial adenomatous polyposis, COX-2 = cyclooxygenase-2, IHC = immunohistochemistry\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eTable 3. Radiological characteristics of the 31 patients with pancreatic desmoid-type fibromatosis\u003c/strong\u003e\u003c/p\u003e\n\u003ctable border=\"1\" cellspacing=\"0\" cellpadding=\"0\" width=\"548\"\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd width=\"100%\" colspan=\"2\" height=\"24\" bgcolor=\"#F2F2F2\"\u003e\n \u003cp\u003eParameters\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eSize (median [range]) (n = 30)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e65.5 mm [19\u0026ndash;284]\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"23\"\u003e\n \u003cp\u003eLocation\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"23\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eHead\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e4/31 (12.9%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eBody\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e4/31 (12.9%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eTail\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e23/31 (74.2%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eMorphology\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"23\"\u003e\n \u003cp\u003eSolid\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"23\"\u003e\n \u003cp\u003e14/31 (45.2%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eCystic\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e8/31 (25.8%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eSolid and cystic\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e9/31 (29.0%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eTumor margin\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eWell-defined\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e14/31 (45.2%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"23\"\u003e\n \u003cp\u003eIll-defined\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"23\"\u003e\n \u003cp\u003e17/31 (54.8%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eCT attenuation of solid portion\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eIntermediate\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e5/5 (100%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eCT attenuation of cystic portion\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eIntermediate\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e1/5 (20%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"23\"\u003e\n \u003cp\u003eLow\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"23\"\u003e\n \u003cp\u003e4/5 (80%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eCT enhancement of solid portion\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eHomogeneous\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e7/20 (35%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eHeterogeneous\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e13/20 (65%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eIn the arterial phase\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"23\"\u003e\n \u003cp\u003eWeak\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"23\"\u003e\n \u003cp\u003e9/9 (100%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eIn the late phase\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eStrong\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e1/17 (5.9%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eModerate\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e8/17 (47.1%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eWeak\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e8/17 (47.1%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"23\"\u003e\n \u003cp\u003eT2WI signal intensity of solid portion\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"23\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eHigh\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e2/4 (50%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eIntermediate\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e2/4 (50%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eFat-suppressed T2WI signal intensity of solid portion\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eHigh\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e1/4 (25%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"23\"\u003e\n \u003cp\u003eIntermediate\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"23\"\u003e\n \u003cp\u003e3/4 (75%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eT2WI signal intensity of cystic portion\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eHigh\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e2/2 (100%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eFat-suppressed T2WI signal intensity of cystic portion\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eHigh\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e2/2 (100%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"23\"\u003e\n \u003cp\u003eT1WI signal intensity of solid portion\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"23\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eHigh\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e1/3 (33.3%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eIntermediate\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e2/3 (66.7%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eFat-suppressed T1WI signal intensity of solid portion\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"23\"\u003e\n \u003cp\u003eHigh\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"23\"\u003e\n \u003cp\u003e1/2 (50%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eIntermediate\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e1/2 (50%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eT1WI signal intensity of cystic portion\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eIntermediate\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e1/2 (50%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eLow\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e1/2 (50%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"23\"\u003e\n \u003cp\u003eFat-suppressed T1WI signal intensity of cystic portion\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"23\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eHigh\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e1/1 (100%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eHemorrhage\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e1/31 (3.2%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eMRI enhancement of solid portion\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eHomogeneous\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e2/3 (66.7%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"23\"\u003e\n \u003cp\u003eHeterogeneous\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"23\"\u003e\n \u003cp\u003e1/3 (33.3%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eIn the arterial phase\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eWeak\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e1/1 (100%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eIn the late phase\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eStrong\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e2/3 (66.7%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eWeak\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e1/3 (33.3%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"23\"\u003e\n \u003cp\u003eDiffusion restriction\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"23\"\u003e\n \u003cp\u003e1/2 (50%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eFDG uptake on PET/CT\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eAvid\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e2/2 (100%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"68.97810218978103%\" height=\"24\"\u003e\n \u003cp\u003eNo metastasis\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"31.021897810218977%\" height=\"24\"\u003e\n \u003cp\u003e7/7 (100%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n\u003c/table\u003e\n\u003cp\u003eCT: computed tomography, T2WI: T2-weighted imaging, T1WI: T1-weighted imaging, MRI: magnetic resonance imaging, FDG: F-18 fluorodeoxyglucose, PET: positron emission tomography\u003c/p\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":false,"highlight":"","institution":"","isAcceptedByJournal":true,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"abdominal-radiology","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"aima","sideBox":"Learn more about [Abdominal Radiology](http://link.springer.com/journal/261)","snPcode":"261","submissionUrl":"https://submission.springernature.com/new-submission/261/3","title":"Abdominal Radiology","twitterHandle":"","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"stoa","reportingPortfolio":"Springer Hybrid","inReviewEnabled":true,"inReviewRevisionsEnabled":false},"keywords":"desmoid, pancreatic tumor, systematic review, computed tomography, magnetic resonance imaging","lastPublishedDoi":"10.21203/rs.3.rs-4765339/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-4765339/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003ch2\u003ePurpose\u003c/h2\u003e \u003cp\u003eThis retrospective study aimed to investigate the radiological features of pancreatic desmoid-type fibromatosis (PDF) and systematically review the previous publications and two new cases.\u003c/p\u003e\u003ch2\u003eMethods\u003c/h2\u003e \u003cp\u003eWe searched PubMed, Cochrane Library, and Web of Science Core Collection and included 31 patients with pathologically proven PDFs with analyzable preoperative computed tomography (CT) and magnetic resonance imaging, including two patients from our institution and 29 patients from 28 publications. Two board-certified radiologists reviewed all images.\u003c/p\u003e\u003ch2\u003eResults\u003c/h2\u003e \u003cp\u003eThe median age of the patients was 39 years, with a male dominance observed (male, 54.8% vs. female, 45.2%). Abdominal pain was the most frequent symptom, occurring in 58.1% of cases. Surgical resection was performed in all cases of PDFs, resulting in a recurrence rate of 8.3% (2/24). The tumors were most commonly located in the pancreatic tail (23/31, 74.2%). In terms of morphology, a \"solid\" shape was most prevalent (14/31, 45.2%), followed by a \"solid and cystic\" shape (9/31, 29.0%) and a \"cystic\" shape (8/31, 25.8%). Characteristic radiological features included heterogeneous enhancement of the solid portion of the tumors on CT scans (13/20, 65%), moderate-to-weak enhancement in the late phase on CT (16/17, 94.1%), and a presence of cystic components in the tumors (17/31, 54.8%). In 16.1% (5/31) of PDFs, the cystic component was pathologically confirmed to be a dilated pancreatic duct.\u003c/p\u003e\u003ch2\u003eConclusion\u003c/h2\u003e \u003cp\u003eWe summarized the clinical and imaging characteristics of PDF. Although the incidence may not be high, cystic components suggesting a dilated pancreatic duct within the tumor are unique imaging features in PDF.\u003c/p\u003e","manuscriptTitle":"Radiological features of pancreatic desmoid-type fibromatosis: A case series and systematic review","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2024-08-17 02:34:38","doi":"10.21203/rs.3.rs-4765339/v1","editorialEvents":[{"type":"communityComments","content":0},{"type":"decision","content":"Revision requested","date":"2024-08-06T11:58:33+00:00","index":"","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2024-08-06T08:06:54+00:00","index":"hide","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2024-08-02T16:01:52+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"36928147306634252676086610131938504032","date":"2024-07-26T08:41:21+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"23897034958195264232582869462246872848","date":"2024-07-24T16:14:13+00:00","index":"hide","fulltext":""},{"type":"reviewersInvited","content":"","date":"2024-07-24T05:43:38+00:00","index":"","fulltext":""},{"type":"editorAssigned","content":"","date":"2024-07-19T02:23:33+00:00","index":"","fulltext":""},{"type":"checksComplete","content":"","date":"2024-07-19T02:23:07+00:00","index":"","fulltext":""},{"type":"submitted","content":"Abdominal Radiology","date":"2024-07-18T23:53:58+00:00","index":"","fulltext":""}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"abdominal-radiology","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"aima","sideBox":"Learn more about [Abdominal Radiology](http://link.springer.com/journal/261)","snPcode":"261","submissionUrl":"https://submission.springernature.com/new-submission/261/3","title":"Abdominal Radiology","twitterHandle":"","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"stoa","reportingPortfolio":"Springer Hybrid","inReviewEnabled":true,"inReviewRevisionsEnabled":false}}],"origin":"","ownerIdentity":"1fe856b4-cbd5-4e8b-9d97-72da17a7f5a9","owner":[],"postedDate":"August 17th, 2024","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"published-in-journal","subjectAreas":[],"tags":[],"updatedAt":"2024-09-16T16:04:15+00:00","versionOfRecord":{"articleIdentity":"rs-4765339","link":"https://doi.org/10.1007/s00261-024-04570-8","journal":{"identity":"abdominal-radiology","isVorOnly":false,"title":"Abdominal Radiology"},"publishedOn":"2024-09-15 15:58:00","publishedOnDateReadable":"September 15th, 2024"},"versionCreatedAt":"2024-08-17 02:34:38","video":"","vorDoi":"10.1007/s00261-024-04570-8","vorDoiUrl":"https://doi.org/10.1007/s00261-024-04570-8","workflowStages":[]},"version":"v1","identity":"rs-4765339","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-4765339","identity":"rs-4765339","version":["v1"]},"buildId":"qtupq5eGEP_6zYnWcrvyt","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}