Cellular angiofibroma of the female pelvic cavity: a case report

Cellular angiofibroma of the female pelvic cavity: a case report | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Case Report Cellular angiofibroma of the female pelvic cavity: a case report Li Chen, Gumuyang Zhang, Hao Sun This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-4685593/v1 This work is licensed under a CC BY 4.0 License Status: Published Journal Publication published 22 Aug, 2024 Read the published version in BMC Urology → Version 1 posted 11 You are reading this latest preprint version Abstract Background: Cellular angiofibroma is a rare benign mesenchymal tumor that mostly occurs in the genital area. Its occurrence outside this region, particularly in the pelvis, is extremely rare. To our knowledge, this study reports the first case of cellular angiofibroma occurring in the pelvic cavity, except for one case reported in the retroperitoneum. Case presentation: A 25-year-old female patient with chronic, intermittent, dull pain in the lower abdomen that lasted for several months was referred to our clinic. Imaging studies revealed a tumor in the pelvic cavity anterior to the bladder. The radiographic characteristics of this tumor indicated a hypervascular nature, suggesting the possibility of a pheochromocytoma or a neuroendocrine tumor. The patient underwent surgical excision of the lesion. To date, no recurrence has been observed three months after excision. Conclusions: Cellular angiofibroma, although rare in the pelvic cavity, should be considered in the differential diagnosis of hypervascular pelvic space-occupying lesions. Immunohistochemical staining can help confirm the diagnosis of this condition. Treatment is generally straightforward, involving local excision of the tumor followed by postoperative monitoring. Cellular angiofibroma mesenchymal tumor p16 case report Figures Figure 1 Figure 2 Figure 3 Introduction Cellular angiofibroma (CA) is a rare benign mesenchymal tumor that was first documented by Nucci et al. in 1997 [ 1 ] . It predominantly occurs in the genital area. CA commonly affects females in the vulva and vagina, while it affects males in the groin and scrotum. Although CA is rarely found in the pelvis, it has been reported in the retroperitoneum [ 2 – 4 ] . CA affects both genders, but it is more common in women, particularly those in their fifties [ 5 ] . The tumor manifests as a gradually growing mass and is often asymptomatic, although it may cause pain in some patients [ 6 ] . In this report, we present a rare and clinically atypical case of a 25-year-old woman with CA located anterior to the bladder in the pelvis. Additionally, we detailed the characteristics, imaging findings, histopathologic features, and treatment of the patient. To our knowledge, this is the second reported case of cellular angiofibroma occurring in a female pelvis. Case Report This case report was approved for publication by the institutional review board. All of the patient details were anonymized to ensure patient confidentiality. A 25-year-old female patient with intermittent, dull pain in the lower abdomen for several months was referred to our outpatient clinic. She had regular menstrual cycles with no significant past medical or gynecological history. The patient underwent computed tomography urography (CTU) in our clinic, along with a complete blood count, blood pressure and glucose monitoring, a 24-hour catecholamine assay, and biochemical examinations for tumor marker profiling. The results of these laboratory tests were within the normal range, and tumor markers, such as carbohydrate antigen (CA) 125, CA19-9, carcinoembryonic antigen (CEA), squamous cell carcinoma antigen, neuron-specific enolase, and lactate dehydrogenase (LDH)-199 were also within the established normal range. The CTU revealed an elliptical soft tissue density in the pelvic cavity, anterior to the bladder, measuring approximately 50 mm × 38 mm × 35 mm. The lesion had a relatively well-defined border with heterogeneous density on non-contrast imaging, characterized by multiple small cystic low-density areas. In addition, its solid component had a CT value of approximately 56 Hounsfield units (HU). During the arterial phase of the contrast-enhanced scanning, the lesion was significantly enhanced with a CT value of 149 HU, particularly within the small cystic areas. It was also significantly enhanced at the tail-like projections. In the portal venous and delayed phases, sustained enhancement with slightly reduced intensity was observed in the lesion, with multiple tortuous and dilated arteries and veins surrounding it. The CT values of the lesion were approximately 122 HU and 101 HU during the venous and delayed phases, respectively. This mass was found close to the anterior wall of the bladder, clearly demarcated from the bladder and adjacent bowel. The mass effect caused a posterior displacement of the anterior bladder wall (Figs. 1 – 2 ). Based on these findings, our institution's preliminary diagnosis was pheochromocytoma or neuroendocrine tumor. Consequently, the patient underwent a complete surgical excision of the mass. Microscopic examination of the specimen showed a spindle cell tumor with dense cellularity, abundant eosinophilic cytoplasm, mild nuclear atypia, and a highly vascularized stroma with areas of hyaline degeneration. Immunohistochemical (IHC) staining revealed cytoplasmic positivity for CD34, smooth muscle actin (SMA), and β-catenin, while desmin, CD117, S-100, and DOG-1 were negative, with a Ki-67 index of 5% (Fig. 3 ). Based on the immunohistochemical results, the final diagnosis was confirmed as CA, and no tumor recurrence was observed during the three-month postoperative follow-up period. Discussion Although studies have reported tumors occurring in the genital region, the etiology of CA is not fully understood [ 7 ] . Lane et al. reported a possible link between long-term estrogen therapy and CA development [ 8 ] . Some researchers have also proposed that CA arises from the differentiation of fibroblasts or myofibroblasts. Fluorescence in situ hybridization and other analytical methods have been used to reveal deletions in the chromosomal region 13q14 in cases of CA, spindle cell lipomas, and mammary and vaginal myofibromatous tumors [ 9 ] . The similar histopathological features observed in this study suggest a potential link between these tumors [ 10 ] . Although CA mainly affects the vulvar, vaginal, and scrotal regions, rare cases have been reported in the oral mucosa, male pelvis, subcutaneous tissue of the chest wall, nasopharynx, retroperitoneum, and anorectal region [ 11 – 15 ] . CA is a well-circumscribed benign mesenchymal tumor, often located in the superficial soft tissues of the trunk [ 6 ] . Most cases of CA present as asymptomatic subcutaneous nodules, except for those in the nasopharynx and retroperitoneum. In the nasopharyngeal region, the tumor manifests as frequent epistaxis (nosebleeds) and persistent nasal congestion [ 12 ] , whereas retroperitoneal tumors cause pain in the iliac fossa [ 2 ] . In this report, we presented the second known case of CA in a female pelvis with atypical clinical characteristics, manifesting only as mild lower abdominal pain. The histopathological changes in CA include the presence of a well-circumscribed tumor located within the dermis and lacking a capsule [ 7 ] . In most patients, the neoplastic cellular component is predominant, with short, bluntly rounded spindle-shaped nuclei and sparse cytoplasm with indistinct cell borders. Additionally, medium-sized, thick-walled blood vessels with hyaline degeneration, slender collagen fiber bundles, and enlarged histiocytic cells are prevalent in this condition. Pseudo-vascular clefts may occasionally be observed, and mature adipocytes are often present. Similar to other tumors, focal cellular atypia was described in this report, and sarcomatous changes were also identified. IHC revealed that the tumor cells exhibited diffuse vimentin positivity, and CD34 was positive in most cases [ 7 ] . In female patients, some tumor cells showed partial positivity for estrogen receptor and progesterone receptor, while SMA, desmin, and S-100 were generally negative. However, in male patients, SMA, muscle-specific actin, and desmin may show partial positivity. The Ki-67 proliferation index is low. CA often appears as a well-circumscribed, hypervascular tumor on CT or MRI images, with no other specific radiographic findings. It requires differentiation from the following conditions. ① Spindle cell lipoma: This tumor predominantly occurs in the posterior neck, shoulder, and upper back of males. The tumor comprises mature adipocytes and slender spindle cells in varying proportions, with myxoid degeneration in the stroma forming pseudo-vascular spaces. Spindle cells are CD34 positive, and mature adipocytes are S-100 positive. ② Angiomatoid fibrous histiocytoma: A soft tissue tumor that commonly occurs in the external genitalia and perineal region, histologically characterized by multi-cellular and hypocellular areas, with prominent myxoid changes in the hypocellular areas. The tumor cells are epithelioid or spindle-shaped, clustered around blood vessels, and often multinucleated, with transparent or pink cytoplasm. They show strong positivity for desmin and vimentin. ③ Retroperitoneal neurofibroma: It is located along the sympathetic ganglia in the paravertebral region. These tumors are well-circumscribed and variable in size, showing positivity for S-100. ④ Aggressive angiomyxoma: This tumor, often 10 cm or larger in diameter, exhibits infiltrative growth. It is composed of small spindle or stellate cells with lightly stained cytoplasm set within a myxoid matrix. These tumor cells express SMA and desmin; ⑤ Labial Leiomyoma: This tumor consists of fascicles of spindle cells with well-defined cytoplasmic borders and cigar-shaped nuclei, which are positive for SMA [ 16 ] . Diagnosing CA based on clinical symptoms and imaging findings before surgery is challenging due to the absence of specific signs. Definitive diagnosis depends on needle biopsy or surgical excision followed by pathological examination. However, CT or MRI can indicate the extent of the tumor and guide the best surgical approach for complete lesion removal. In summary, CA, although rare in the pelvic cavity, should be considered in the differential diagnosis of hypervascular pelvic space-occupying lesions. Most cases of CA exhibit benign behavior and are easily excised without showing atypia, and follow-up studies show low recurrence and no metastatic potential [ 17 – 19 ] . However, Hanae et al. suggested that previous research might have underestimated the recurrence or metastasic potential of CA, particularly in specific populations such as pregnant women, emphasizing the need for long-term follow-up studies [ 2 ] . The limitation in this case is the short follow-up period. Declarations Ethics approval and consent to participate The Pecking Union College Hospital Institutional Review Board approval was obtained. Written informed consent was obtained from all participants before their inclusion in the study. Consent for publication Written informed consent for publication was obtained from the patient for their personal or clinical details along with any identifying images to be published in this study. Availability of data and materials The data used during the current study are available from the corresponding author of reasonable request. Competing interests All the authors declare they have no competing interests. Funding None. Authors' contributions Li Chen and Gumuyang Zhang contributed to writing original draft preparation. Hao Sun contributed to the idea design of the paper and the modification of the final draft. All authors read and approved the final manuscript. Acknowledgments We thank LetPub (www.letpub.com.cn) for its linguistic assistance during the preparation of this manuscript. References NUCCI M R, GRANTER S R, FLETCHER C D. Cellular angiofibroma: a benign neoplasm distinct from angiomyofibroblastoma and spindle cell lipoma [J]. Am J Surg Pathol, 1997, 21(6): 636-44. ABE H, NAKAO S, ITAGAKI H, et al. Cellular angiofibroma in the retroperitoneal space during pregnancy: A case report [J]. BMC Womens Health, 2023, 23(1): 57. ECKHARDT S, ROLSTON R, PALMER S, et al. Vaginal Angiomyofibroblastoma: A Case Report and Review of Diagnostic Imaging [J]. Case Rep Obstet Gynecol, 2018, 2018: 7397121. VAN MULDERS S, FAES E, BROECKX G, et al. Cervicovaginal cellular angiofibroma [J]. BMJ Case Rep, 2020, 13(7). HLAING T, TSE G. Angiomyofibroblastoma of the Male Perineum: An Unusual Location for a Rare Lesion [J]. Int J Surg Pathol, 2000, 8(1): 79-82. CHEN E, FLETCHER C D. Cellular angiofibroma with atypia or sarcomatous transformation: clinicopathologic analysis of 13 cases [J]. Am J Surg Pathol, 2010, 34(5): 707-14. IWASA Y, FLETCHER C D. Cellular angiofibroma: clinicopathologic and immunohistochemical analysis of 51 cases [J]. Am J Surg Pathol, 2004, 28(11): 1426-35. LANE J E, WALKER A N, MULLIS E N, JR., et al. Cellular angiofibroma of the vulva [J]. Gynecol Oncol, 2001, 81(2): 326-9. PTASZYNSKI K, SZUMERA-CIECKIEWICZ A, BARTCZAK A. Cellular angiofibroma with atypia or sarcomatous transformation - case description with literature review [J]. Pol J Pathol, 2012, 63(3): 207-11. PANAGOPOULOS I, GORUNOVA L, BJERKEHAGEN B, et al. Loss of chromosome 13 material in cellular angiofibromas indicates pathogenetic similarity with spindle cell lipomas [J]. Diagn Pathol, 2017, 12(1): 17. ERDUR Z B, YENER H M, YILMAZ M, et al. Cellular Angiofibroma of the Nasopharynx [J]. J Craniofac Surg, 2017, 28(8): e720-e2. LIU Y, XU Y, WANG Q, et al. Cellular angiofibroma in the hypopharynx: A case report [J]. Medicine (Baltimore), 2019, 98(50): e18385. MATSUMOTO N P, AHMED M, ROTTMANN D, et al. Cellular angiofibroma arising in the anorectal region: clinicopathologic and immunohistochemical analysis of five cases [J]. Hum Pathol, 2022, 120: 1-8. BRANDAO A, CAMPOS S, FRAGA J, et al. [Retroperitoneal Cellular Angiofibroma: A Rare Gynecological Entity] [J]. Acta Med Port, 2017, 30(12): 882-6. EVERSOLE L R. Cellular angiofibroma of oral mucosa: report of two cases [J]. Head Neck Pathol, 2009, 3(2): 136-9. MANDATO V D, SANTAGNI S, CAVAZZA A, et al. Cellular angiofibroma in women: a review of the literature [J]. Diagn Pathol, 2015, 10: 114. SUN Q, RYU J, KIM Y. Giant cell angiofibroma of gingiva in tuberous sclerosis complex: a case report and literature review [J]. Diagn Pathol, 2024, 19(1): 50. MURASHIMA T, KIDA K, GI T, et al. Paratesticular cellular angiofibroma: a case report [J]. J Med Case Rep, 2024, 18(1): 170. GASPAR REIS S, ALVES D G, ANACLETO S, et al. Paratesticular Cellular Angiofibroma (CAF): A Rare Case Report [J]. Cureus, 2023, 15(8): e43124. Additional Declarations No competing interests reported. Cite Share Download PDF Status: Published Journal Publication published 22 Aug, 2024 Read the published version in BMC Urology → Version 1 posted Editorial decision: Revision requested 23 Jul, 2024 Reviews received at journal 22 Jul, 2024 Reviewers agreed at journal 22 Jul, 2024 Reviewers agreed at journal 21 Jul, 2024 Reviews received at journal 15 Jul, 2024 Reviewers agreed at journal 13 Jul, 2024 Reviewers invited by journal 11 Jul, 2024 Editor invited by journal 10 Jul, 2024 Editor assigned by journal 10 Jul, 2024 Submission checks completed at journal 10 Jul, 2024 First submitted to journal 04 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. 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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-4685593","acceptedTermsAndConditions":true,"allowDirectSubmit":false,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":330866469,"identity":"3582e285-bd33-44b6-b0a3-eef34bb8d95c","order_by":0,"name":"Li Chen","email":"","orcid":"","institution":"Peking Union Medical College Hospital","correspondingAuthor":false,"prefix":"","firstName":"Li","middleName":"","lastName":"Chen","suffix":""},{"id":330866470,"identity":"c3169784-8181-4234-9f6a-c79cffb701da","order_by":1,"name":"Gumuyang Zhang","email":"","orcid":"","institution":"Peking Union Medical College Hospital","correspondingAuthor":false,"prefix":"","firstName":"Gumuyang","middleName":"","lastName":"Zhang","suffix":""},{"id":330866472,"identity":"a76aa28b-47bf-40b1-ae8c-1406bb539c72","order_by":2,"name":"Hao Sun","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAA30lEQVRIiWNgGAWjYHACNgglASIqJOT4SdNy4IyFsWQDSVoOtlUkbiCkRX5G7rEHPyoY8vmlm499/jhPgnEDA/PDRzfwaDG4kZdu2HOGwXLmnGPJMw5uk2A2Z2AzNs7Bp0Uix0yCt43BwOBGjjEDUAubZQMPmzQ+LfIzcswk/4K15H9mODhHgsfgAAEtDDdyzKShtjAzHGyQkCCoxeDMGzNpmTMMBpJzjhkznDkmYSDZTMAv8u1Ah72pYDAAhthjhoqauvp+9uaHj/E6DAL+I7GZCSsfBaNgFIyCUUAAAACT6kT2KrMgDAAAAABJRU5ErkJggg==","orcid":"","institution":"Peking Union Medical College Hospital","correspondingAuthor":true,"prefix":"","firstName":"Hao","middleName":"","lastName":"Sun","suffix":""}],"badges":[],"createdAt":"2024-07-04 09:52:05","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-4685593/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-4685593/v1","draftVersion":[],"editorialEvents":[{"content":"https://doi.org/10.1186/s12894-024-01566-w","type":"published","date":"2024-08-22T15:57:17+00:00"}],"editorialNote":"","failedWorkflow":false,"files":[{"id":62185634,"identity":"c8702c77-b857-4693-9aff-f4a6a294685f","added_by":"auto","created_at":"2024-08-10 11:55:09","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":1099590,"visible":true,"origin":"","legend":"\u003cp\u003eThe depicted images are as follows: (A) the non-contrast phase, (B) the arterial phase, (C) the portal venous phase, and (D) the delayed phase. The tumor exhibits a well-defined border with a heterogeneous composition. Its solid components were significantly enhanced during the contrast-enhanced scans, with a slight decrease in enhancement intensity over the phases.\u003c/p\u003e","description":"","filename":"Figure1..png","url":"https://assets-eu.researchsquare.com/files/rs-4685593/v1/10ac0711edd39e4841bc140d.png"},{"id":62185635,"identity":"90d99b4a-922c-4dac-b1e9-ca3a77318e0d","added_by":"auto","created_at":"2024-08-10 11:55:10","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":1072827,"visible":true,"origin":"","legend":"\u003cp\u003e(A) Coronal reformatted imaging of the tumor clearly delineated from the surrounding bowel and bladder, exerting significant pressure on them. (B) Volume rendering technique reconstruction reveals abundant vascularity within the tumor.\u003c/p\u003e","description":"","filename":"Figure2..png","url":"https://assets-eu.researchsquare.com/files/rs-4685593/v1/5a8e4c224317a7dc584f299f.png"},{"id":62185636,"identity":"aa8610fc-da36-4da6-9fc9-0eef12118f34","added_by":"auto","created_at":"2024-08-10 11:55:10","extension":"png","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":4261567,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cstrong\u003eA\u003c/strong\u003e(magnification ×250): the hematoxylin-eosin staining specimen shows a spindle cell tumor with dense cellularity, abundant eosinophilic cytoplasm, mild nuclear atypia, and a highly vascularized stroma, with areas of hyaline degeneration. \u003cstrong\u003eB \u003c/strong\u003e(magnification ×100): immunohistochemical staining reveals cytoplasmic positivity for CD34. \u003cstrong\u003eC \u003c/strong\u003e(magnification ×100):\u003cstrong\u003e \u003c/strong\u003eIHC staining revealed cytoplasmic positivity for β-catenin. \u003cstrong\u003eD\u003c/strong\u003e (magnification ×100): IHC staining revealed negative for S-100.\u003c/p\u003e","description":"","filename":"Figure3..png","url":"https://assets-eu.researchsquare.com/files/rs-4685593/v1/65be5fe8350ed7a02dab2f88.png"},{"id":63300170,"identity":"925ba724-d419-4f4a-a9fb-ef9a2ef1662d","added_by":"auto","created_at":"2024-08-26 16:12:11","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":6746784,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-4685593/v1/3d9b0ce4-de75-45cc-9370-e554f842ea5a.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Cellular angiofibroma of the female pelvic cavity: a case report","fulltext":[{"header":"Introduction","content":"\u003cp\u003eCellular angiofibroma (CA) is a rare benign mesenchymal tumor that was first documented by Nucci et al. in 1997 \u003csup\u003e[\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e]\u003c/sup\u003e. It predominantly occurs in the genital area. CA commonly affects females in the vulva and vagina, while it affects males in the groin and scrotum. Although CA is rarely found in the pelvis, it has been reported in the retroperitoneum \u003csup\u003e[\u003cspan additionalcitationids=\"CR3\" citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e]\u003c/sup\u003e. CA affects both genders, but it is more common in women, particularly those in their fifties \u003csup\u003e[\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e]\u003c/sup\u003e. The tumor manifests as a gradually growing mass and is often asymptomatic, although it may cause pain in some patients \u003csup\u003e[\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e]\u003c/sup\u003e.\u003c/p\u003e \u003cp\u003eIn this report, we present a rare and clinically atypical case of a 25-year-old woman with CA located anterior to the bladder in the pelvis. Additionally, we detailed the characteristics, imaging findings, histopathologic features, and treatment of the patient. To our knowledge, this is the second reported case of cellular angiofibroma occurring in a female pelvis.\u003c/p\u003e"},{"header":"Case Report","content":"\u003cp\u003eThis case report was approved for publication by the institutional review board. All of the patient details were anonymized to ensure patient confidentiality.\u003c/p\u003e \u003cp\u003eA 25-year-old female patient with intermittent, dull pain in the lower abdomen for several months was referred to our outpatient clinic. She had regular menstrual cycles with no significant past medical or gynecological history.\u003c/p\u003e \u003cp\u003eThe patient underwent computed tomography urography (CTU) in our clinic, along with a complete blood count, blood pressure and glucose monitoring, a 24-hour catecholamine assay, and biochemical examinations for tumor marker profiling. The results of these laboratory tests were within the normal range, and tumor markers, such as carbohydrate antigen (CA) 125, CA19-9, carcinoembryonic antigen (CEA), squamous cell carcinoma antigen, neuron-specific enolase, and lactate dehydrogenase (LDH)-199 were also within the established normal range. The CTU revealed an elliptical soft tissue density in the pelvic cavity, anterior to the bladder, measuring approximately 50 mm \u0026times; 38 mm \u0026times; 35 mm. The lesion had a relatively well-defined border with heterogeneous density on non-contrast imaging, characterized by multiple small cystic low-density areas. In addition, its solid component had a CT value of approximately 56 Hounsfield units (HU). During the arterial phase of the contrast-enhanced scanning, the lesion was significantly enhanced with a CT value of 149 HU, particularly within the small cystic areas. It was also significantly enhanced at the tail-like projections. In the portal venous and delayed phases, sustained enhancement with slightly reduced intensity was observed in the lesion, with multiple tortuous and dilated arteries and veins surrounding it. The CT values of the lesion were approximately 122 HU and 101 HU during the venous and delayed phases, respectively. This mass was found close to the anterior wall of the bladder, clearly demarcated from the bladder and adjacent bowel. The mass effect caused a posterior displacement of the anterior bladder wall (Figs.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003e\u0026ndash;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003e). Based on these findings, our institution's preliminary diagnosis was pheochromocytoma or neuroendocrine tumor. Consequently, the patient underwent a complete surgical excision of the mass.\u003c/p\u003e \u003cp\u003eMicroscopic examination of the specimen showed a spindle cell tumor with dense cellularity, abundant eosinophilic cytoplasm, mild nuclear atypia, and a highly vascularized stroma with areas of hyaline degeneration. Immunohistochemical (IHC) staining revealed cytoplasmic positivity for CD34, smooth muscle actin (SMA), and β-catenin, while desmin, CD117, S-100, and DOG-1 were negative, with a Ki-67 index of 5% (Fig.\u0026nbsp;\u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e3\u003c/span\u003e). Based on the immunohistochemical results, the final diagnosis was confirmed as CA, and no tumor recurrence was observed during the three-month postoperative follow-up period.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003e \u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003eAlthough studies have reported tumors occurring in the genital region, the etiology of CA is not fully understood \u003csup\u003e[\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]\u003c/sup\u003e. Lane et al. reported a possible link between long-term estrogen therapy and CA development \u003csup\u003e[\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e]\u003c/sup\u003e. Some researchers have also proposed that CA arises from the differentiation of fibroblasts or myofibroblasts. Fluorescence in situ hybridization and other analytical methods have been used to reveal deletions in the chromosomal region 13q14 in cases of CA, spindle cell lipomas, and mammary and vaginal myofibromatous tumors \u003csup\u003e[\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e]\u003c/sup\u003e. The similar histopathological features observed in this study suggest a potential link between these tumors \u003csup\u003e[\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e]\u003c/sup\u003e.\u003c/p\u003e \u003cp\u003eAlthough CA mainly affects the vulvar, vaginal, and scrotal regions, rare cases have been reported in the oral mucosa, male pelvis, subcutaneous tissue of the chest wall, nasopharynx, retroperitoneum, and anorectal region \u003csup\u003e[\u003cspan additionalcitationids=\"CR12 CR13 CR14\" citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR15\" class=\"CitationRef\"\u003e15\u003c/span\u003e]\u003c/sup\u003e. CA is a well-circumscribed benign mesenchymal tumor, often located in the superficial soft tissues of the trunk \u003csup\u003e[\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e]\u003c/sup\u003e. Most cases of CA present as asymptomatic subcutaneous nodules, except for those in the nasopharynx and retroperitoneum. In the nasopharyngeal region, the tumor manifests as frequent epistaxis (nosebleeds) and persistent nasal congestion \u003csup\u003e[\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e]\u003c/sup\u003e, whereas retroperitoneal tumors cause pain in the iliac fossa \u003csup\u003e[\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e]\u003c/sup\u003e. In this report, we presented the second known case of CA in a female pelvis with atypical clinical characteristics, manifesting only as mild lower abdominal pain.\u003c/p\u003e \u003cp\u003eThe histopathological changes in CA include the presence of a well-circumscribed tumor located within the dermis and lacking a capsule \u003csup\u003e[\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]\u003c/sup\u003e. In most patients, the neoplastic cellular component is predominant, with short, bluntly rounded spindle-shaped nuclei and sparse cytoplasm with indistinct cell borders. Additionally, medium-sized, thick-walled blood vessels with hyaline degeneration, slender collagen fiber bundles, and enlarged histiocytic cells are prevalent in this condition. Pseudo-vascular clefts may occasionally be observed, and mature adipocytes are often present. Similar to other tumors, focal cellular atypia was described in this report, and sarcomatous changes were also identified. IHC revealed that the tumor cells exhibited diffuse vimentin positivity, and CD34 was positive in most cases \u003csup\u003e[\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]\u003c/sup\u003e. In female patients, some tumor cells showed partial positivity for estrogen receptor and progesterone receptor, while SMA, desmin, and S-100 were generally negative. However, in male patients, SMA, muscle-specific actin, and desmin may show partial positivity. The Ki-67 proliferation index is low.\u003c/p\u003e \u003cp\u003eCA often appears as a well-circumscribed, hypervascular tumor on CT or MRI images, with no other specific radiographic findings. It requires differentiation from the following conditions. ① Spindle cell lipoma: This tumor predominantly occurs in the posterior neck, shoulder, and upper back of males. The tumor comprises mature adipocytes and slender spindle cells in varying proportions, with myxoid degeneration in the stroma forming pseudo-vascular spaces. Spindle cells are CD34 positive, and mature adipocytes are S-100 positive. ② Angiomatoid fibrous histiocytoma: A soft tissue tumor that commonly occurs in the external genitalia and perineal region, histologically characterized by multi-cellular and hypocellular areas, with prominent myxoid changes in the hypocellular areas. The tumor cells are epithelioid or spindle-shaped, clustered around blood vessels, and often multinucleated, with transparent or pink cytoplasm. They show strong positivity for desmin and vimentin. ③ Retroperitoneal neurofibroma: It is located along the sympathetic ganglia in the paravertebral region. These tumors are well-circumscribed and variable in size, showing positivity for S-100. ④ Aggressive angiomyxoma: This tumor, often 10 cm or larger in diameter, exhibits infiltrative growth. It is composed of small spindle or stellate cells with lightly stained cytoplasm set within a myxoid matrix. These tumor cells express SMA and desmin; ⑤ Labial Leiomyoma: This tumor consists of fascicles of spindle cells with well-defined cytoplasmic borders and cigar-shaped nuclei, which are positive for SMA \u003csup\u003e[\u003cspan citationid=\"CR16\" class=\"CitationRef\"\u003e16\u003c/span\u003e]\u003c/sup\u003e.\u003c/p\u003e \u003cp\u003eDiagnosing CA based on clinical symptoms and imaging findings before surgery is challenging due to the absence of specific signs. Definitive diagnosis depends on needle biopsy or surgical excision followed by pathological examination. However, CT or MRI can indicate the extent of the tumor and guide the best surgical approach for complete lesion removal.\u003c/p\u003e \u003cp\u003eIn summary, CA, although rare in the pelvic cavity, should be considered in the differential diagnosis of hypervascular pelvic space-occupying lesions. Most cases of CA exhibit benign behavior and are easily excised without showing atypia, and follow-up studies show low recurrence and no metastatic potential \u003csup\u003e[\u003cspan additionalcitationids=\"CR18\" citationid=\"CR17\" class=\"CitationRef\"\u003e17\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR19\" class=\"CitationRef\"\u003e19\u003c/span\u003e]\u003c/sup\u003e. However, Hanae et al. suggested that previous research might have underestimated the recurrence or metastasic potential of CA, particularly in specific populations such as pregnant women, emphasizing the need for long-term follow-up studies \u003csup\u003e[\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e]\u003c/sup\u003e. The limitation in this case is the short follow-up period.\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eEthics approval and consent to participate\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe Pecking Union College Hospital Institutional Review Board approval was obtained.\u0026nbsp;Written informed consent was obtained from all participants before their inclusion in the study.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConsent for publication\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eWritten informed consent for publication was obtained from the patient for their personal or clinical details along with any identifying images to be published in this study.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAvailability of data and materials\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe data used during the current study are available from the corresponding author of reasonable request.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCompeting interests\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eAll the authors\u0026nbsp;declare they have no competing interests.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eFunding\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNone.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAuthors\u0026apos; contributions\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eLi Chen and Gumuyang Zhang contributed to writing original draft preparation. Hao Sun contributed to the idea design of the paper and the modification of the final draft.\u003c/p\u003e\n\u003cp\u003eAll authors read and approved the final manuscript.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAcknowledgments\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eWe thank LetPub (www.letpub.com.cn) for its linguistic assistance during the preparation of this manuscript.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n\u003cli\u003eNUCCI M R, GRANTER S R, FLETCHER C D. Cellular angiofibroma: a benign neoplasm distinct from angiomyofibroblastoma and spindle cell lipoma [J]. Am J Surg Pathol, 1997, 21(6): 636-44.\u003c/li\u003e\n\u003cli\u003eABE H, NAKAO S, ITAGAKI H, et al. Cellular angiofibroma in the retroperitoneal space during pregnancy: A case report [J]. BMC Womens Health, 2023, 23(1): 57.\u003c/li\u003e\n\u003cli\u003eECKHARDT S, ROLSTON R, PALMER S, et al. Vaginal Angiomyofibroblastoma: A Case Report and Review of Diagnostic Imaging [J]. Case Rep Obstet Gynecol, 2018, 2018: 7397121.\u003c/li\u003e\n\u003cli\u003eVAN MULDERS S, FAES E, BROECKX G, et al. Cervicovaginal cellular angiofibroma [J]. BMJ Case Rep, 2020, 13(7).\u003c/li\u003e\n\u003cli\u003eHLAING T, TSE G. Angiomyofibroblastoma of the Male Perineum: An Unusual Location for a Rare Lesion [J]. Int J Surg Pathol, 2000, 8(1): 79-82.\u003c/li\u003e\n\u003cli\u003eCHEN E, FLETCHER C D. Cellular angiofibroma with atypia or sarcomatous transformation: clinicopathologic analysis of 13 cases [J]. Am J Surg Pathol, 2010, 34(5): 707-14.\u003c/li\u003e\n\u003cli\u003eIWASA Y, FLETCHER C D. Cellular angiofibroma: clinicopathologic and immunohistochemical analysis of 51 cases [J]. Am J Surg Pathol, 2004, 28(11): 1426-35.\u003c/li\u003e\n\u003cli\u003eLANE J E, WALKER A N, MULLIS E N, JR., et al. Cellular angiofibroma of the vulva [J]. Gynecol Oncol, 2001, 81(2): 326-9.\u003c/li\u003e\n\u003cli\u003ePTASZYNSKI K, SZUMERA-CIECKIEWICZ A, BARTCZAK A. Cellular angiofibroma with atypia or sarcomatous transformation - case description with literature review [J]. Pol J Pathol, 2012, 63(3): 207-11.\u003c/li\u003e\n\u003cli\u003ePANAGOPOULOS I, GORUNOVA L, BJERKEHAGEN B, et al. Loss of chromosome 13 material in cellular angiofibromas indicates pathogenetic similarity with spindle cell lipomas [J]. Diagn Pathol, 2017, 12(1): 17.\u003c/li\u003e\n\u003cli\u003eERDUR Z B, YENER H M, YILMAZ M, et al. Cellular Angiofibroma of the Nasopharynx [J]. J Craniofac Surg, 2017, 28(8): e720-e2.\u003c/li\u003e\n\u003cli\u003eLIU Y, XU Y, WANG Q, et al. Cellular angiofibroma in the hypopharynx: A case report [J]. Medicine (Baltimore), 2019, 98(50): e18385.\u003c/li\u003e\n\u003cli\u003eMATSUMOTO N P, AHMED M, ROTTMANN D, et al. Cellular angiofibroma arising in the anorectal region: clinicopathologic and immunohistochemical analysis of five cases [J]. Hum Pathol, 2022, 120: 1-8.\u003c/li\u003e\n\u003cli\u003eBRANDAO A, CAMPOS S, FRAGA J, et al. [Retroperitoneal Cellular Angiofibroma: A Rare Gynecological Entity] [J]. Acta Med Port, 2017, 30(12): 882-6.\u003c/li\u003e\n\u003cli\u003eEVERSOLE L R. Cellular angiofibroma of oral mucosa: report of two cases [J]. Head Neck Pathol, 2009, 3(2): 136-9.\u003c/li\u003e\n\u003cli\u003eMANDATO V D, SANTAGNI S, CAVAZZA A, et al. Cellular angiofibroma in women: a review of the literature [J]. Diagn Pathol, 2015, 10: 114.\u003c/li\u003e\n\u003cli\u003eSUN Q, RYU J, KIM Y. Giant cell angiofibroma of gingiva in tuberous sclerosis complex: a case report and literature review [J]. Diagn Pathol, 2024, 19(1): 50.\u003c/li\u003e\n\u003cli\u003eMURASHIMA T, KIDA K, GI T, et al. Paratesticular cellular angiofibroma: a case report [J]. J Med Case Rep, 2024, 18(1): 170.\u003c/li\u003e\n\u003cli\u003eGASPAR REIS S, ALVES D G, ANACLETO S, et al. Paratesticular Cellular Angiofibroma (CAF): A Rare Case Report [J]. Cureus, 2023, 15(8): e43124.\u003c/li\u003e\n\u003c/ol\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":"bmc-urology","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"buro","sideBox":"Learn more about [BMC Urology](http://bmcurol.biomedcentral.com/)","snPcode":"","submissionUrl":"https://www.editorialmanager.com/buro/default.aspx","title":"BMC Urology","twitterHandle":"BMC_series","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"em","reportingPortfolio":"BMC Series","inReviewEnabled":true,"inReviewRevisionsEnabled":true},"keywords":"Cellular angiofibroma, mesenchymal tumor, p16, case report","lastPublishedDoi":"10.21203/rs.3.rs-4685593/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-4685593/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003eBackground:\u003c/p\u003e\n\u003cp\u003eCellular angiofibroma is a rare benign mesenchymal tumor that mostly occurs in the genital area. Its occurrence outside this region, particularly in the pelvis, is extremely rare. To our knowledge, this study reports the first case of cellular angiofibroma occurring in the pelvic cavity, except for one case reported in the retroperitoneum.\u003c/p\u003e\n\u003cp\u003eCase presentation:\u003c/p\u003e\n\u003cp\u003eA 25-year-old female patient with chronic, intermittent, dull pain in the lower abdomen that lasted for several months was referred to our clinic. Imaging studies revealed a tumor in the pelvic cavity anterior to the bladder. The radiographic characteristics of this tumor indicated a hypervascular nature, suggesting the possibility of a pheochromocytoma or a neuroendocrine tumor. The patient underwent surgical excision of the lesion. To date, no recurrence has been observed three months after excision.\u003c/p\u003e\n\u003cp\u003eConclusions:\u003c/p\u003e\n\u003cp\u003eCellular angiofibroma, although rare in the pelvic cavity, should be considered in the differential diagnosis of hypervascular pelvic space-occupying lesions. Immunohistochemical staining can help confirm the diagnosis of this condition. Treatment is generally straightforward, involving local excision of the tumor followed by postoperative monitoring.\u003c/p\u003e","manuscriptTitle":"Cellular angiofibroma of the female pelvic cavity: a case report","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2024-08-10 11:55:05","doi":"10.21203/rs.3.rs-4685593/v1","editorialEvents":[{"type":"communityComments","content":0},{"type":"decision","content":"Revision requested","date":"2024-07-23T17:42:28+00:00","index":"","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2024-07-22T10:01:30+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"326432453780903021674352005799345080615","date":"2024-07-22T09:37:44+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"187005226821889783487359203492294262291","date":"2024-07-22T02:28:19+00:00","index":"hide","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2024-07-15T08:05:50+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"281399867043561461020639482872531382849","date":"2024-07-13T09:12:26+00:00","index":"hide","fulltext":""},{"type":"reviewersInvited","content":"","date":"2024-07-11T09:09:22+00:00","index":"","fulltext":""},{"type":"editorInvited","content":"","date":"2024-07-10T07:25:46+00:00","index":"","fulltext":""},{"type":"editorAssigned","content":"","date":"2024-07-10T07:23:01+00:00","index":"","fulltext":""},{"type":"checksComplete","content":"","date":"2024-07-10T07:22:48+00:00","index":"","fulltext":""},{"type":"submitted","content":"BMC Urology","date":"2024-07-04T09:50:41+00:00","index":"","fulltext":""}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"bmc-urology","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"buro","sideBox":"Learn more about [BMC Urology](http://bmcurol.biomedcentral.com/)","snPcode":"","submissionUrl":"https://www.editorialmanager.com/buro/default.aspx","title":"BMC Urology","twitterHandle":"BMC_series","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"em","reportingPortfolio":"BMC Series","inReviewEnabled":true,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"4cc40b0d-94bc-461f-8d51-bc644256f187","owner":[],"postedDate":"August 10th, 2024","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"published-in-journal","subjectAreas":[],"tags":[],"updatedAt":"2024-08-26T16:01:16+00:00","versionOfRecord":{"articleIdentity":"rs-4685593","link":"https://doi.org/10.1186/s12894-024-01566-w","journal":{"identity":"bmc-urology","isVorOnly":false,"title":"BMC Urology"},"publishedOn":"2024-08-22 15:57:17","publishedOnDateReadable":"August 22nd, 2024"},"versionCreatedAt":"2024-08-10 11:55:05","video":"","vorDoi":"10.1186/s12894-024-01566-w","vorDoiUrl":"https://doi.org/10.1186/s12894-024-01566-w","workflowStages":[]},"version":"v1","identity":"rs-4685593","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-4685593","identity":"rs-4685593","version":["v1"]},"buildId":"qtupq5eGEP_6zYnWcrvyt","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}