Imaging features of pediatric hepatic sclerosing epithelial fibrosarcoma

Imaging features of pediatric hepatic sclerosing epithelial fibrosarcoma | 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 Imaging features of pediatric hepatic sclerosing epithelial fibrosarcoma Ghazal Shadmani This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-4694052/v1 This work is licensed under a CC BY 4.0 License Status: Posted Version 1 posted You are reading this latest preprint version Abstract Background : Sclerosing epithelioid fibrosarcoma (SEF) is a rare variant of fibrosarcoma that has been described in fewer than a handful of pediatric patients in the literature and in almost all prior publications focused on pathology. There is no description of the imaging features of hepatic SEF in the literature. Patient presentation : A 17-year-old female with chronic abdominal pain and pruritis was found to have a large solid mass in the right hepatic lobe on ultrasonography (US). Further imaging by contrast-enhanced MRI and FDG PET was performed. A core biopsy of the hepatic mass revealed SFE. Two months later, she underwent liver transplantation. On clinical and imaging follow-up up to 24 months after transplantation, no evidence of recurrence or metastasis was identified. Conclusion : This paper presents the first multimodality imaging features of primary hepatic SEF in an adolescent patient. Pediatrics Oncology Nuclear Medicine & Medical Imaging Sclerosing epithelioid fibrosarcoma (SEF) liver pediatric Figures Figure 1 Figure 2 Figure 3 Figure 4 INTRODUCTION Sclerosing epithelioid fibrosarcoma (SEF) is a rare variant of fibrosarcoma that shows round or oval epithelioid cells within a dense collagenous matrix. Although the histologic appearance of this tumor is benign with either an absent or low mitotic rate, the tendency toward local recurrence and synchronous/metachronous distant metastasis can reach 37% and 80%, respectively, suggesting that it is a clinically aggressive tumor [ 1 ]. SFE mainly occurs in middle-aged adults without a significant sex predilection, with a reported median tumor size at presentation of approximately 8 cm. It is most commonly observed in the soft tissues of the lower extremity, trunk, upper extremity, and head and neck region. The primary sites of metastasis are the lung and bone [ 2 ]. Fewer than a handful of pediatric cases have been described in the literature. The locations of SFE in pediatric patients presented in the literature include the bone[ 3 ], trunk (mediastinum, retroperitoneum, back, abdominal wall)[ 4 ], kidney[ 2 ], and liver[ 5 ]. Diagnosis is challenging due to the rarity of the disease, which is ultimately confirmed by pathology. Although the immunohistochemical and molecular genetic characteristics of pediatric hepatic SEF have been explored previously, no studies have focused on its imaging features. Therefore, the imaging characteristics of SEF remain poorly defined in the literature. The aim of this paper was to review the US, MRI, and FDG PET features of this rare pediatric liver malignancy. To the best of our knowledge, this is the first description of the multimodality imaging features of pediatric liver SEFs. CASE REPORT The patient, which will be discussed, was evaluated at our center, and her parents consented to her case being reported in a medical journal. A 17-year-old female, previously healthy, presented with abdominal pain and pruritus for several months. She had noticed jaundice in her eyes for the last two weeks but denied acholic stools. These symptoms were associated with 10-pound weight loss and fatigue. She was not on any steroids or oral contraceptive pills. Initial laboratory data revealed a total bilirubin level of 4.7 mg/dL (0.0-1.2), alkaline phosphatase level of 390 unit/L (40–130), AST level of 101unit/L (10–45), and ALT level of 162 unit/L (7–45). Abdominal ultrasound (Fig. 1) revealed a large lobulated mixed echogenic mass centered on the right hepatic lobe containing several hypoechoic components with posterior acoustic shadowing without a discernible central scar. Color Doppler showed scant peripheral vascularity without significant internal vascularity. The spleen was moderately to markedly enlarged, with a craniocaudal dimension of approximately 19 cm. Upon further laboratory evaluation, CA 19 − 9 was 68.8 units/mL (Nl: up to 35), and her serum alpha-fetoprotein and beta-HCG levels were normal. Abdominal MRI (Figs. 2–3) revealed a large predominantly hypointense mass on both T1- and T2-weighted images with heterogeneous enhancement on delayed images. There was no signal drop throughout the lesion on out-of-phase images to suggest fat component. No central scar was observed within the hepatic lesion. Mild, regular dilation of peripheral intrahepatic biliary ducts was also identified due to the mass effect on the biliary system. Splenomegaly and collateral vessels were present. There was no tumoral invasion or thrombosis of the inferior vena cava. Liver biopsy confirmed the diagnosis of SEF. An FDG PET CT scan for initial staging showed mild to moderate uptake throughout the hepatic mass without evidence of distant metastatic disease (Fig. 4). Two months after the initial presentation, the patient subsequently underwent an orthotopic liver transplant. On the most recent surveillance imaging, 24 months after transplantation, no findings of disease recurrence were identified, and the serum CA 19 − 9 remained normal. DISCUSSION T2 hypointense appearance and progressive delayed contrast enhancement on MRI are well-known imaging features of fibrous tumors secondary to the high collagenous content of the tumor[ 6 ], both of which are present in this case. Another imaging clue suggesting the dense fibrous nature of the tumor is posterior acoustic shadowing of the hypoechoic components on US. Xia et al. described the imaging features of SFE in an adult patient with pancreatic SFE as T1/T2 hypointense pancreatic mass with progressive centripetal enhancement on delayed images [ 7 ]. Several prior case reports described the T2 hypointense appearance of intracranial metastases, ethmoid sinus, and intramuscular SEF [ 8 – 11 ]. Tomimaru et al. presented the first case of SEF in a 35-year-old patient with a 7 cm right hepatic lobe mass and inferior vena cava invasion at the time of presentation with delayed centripetal enhancement on contrast-enhanced CT images [ 12 ]. This finding is expected considering the high fibrous content. Ding et al. described MRI findings in a 19-year-old patient with SEF of the right fibula as a T1/T2 hypointense mass with faint peripheral contrast enhancement during the arterial phase and progressive enhancement during the venous phase [ 13 ]. SEF has been described as having a very slow growth process based on the observation of superficial soft tissue tumors. In the present case, the presence of cavernous venous formation at the porta hepatis and splenomegaly supported the slow growth of the SFE. The differential consideration in the present case included benign and malignant causes; benign lesions included hepatic adenoma and focal nodular hyperplasia. There was no signal drop on out-of-phase images for an internal fat component. Moreover, no T1 hyperintense foci of hemorrhage or central scarring were identified on T1-weighted images. The malignant differential considerations in this patient included metastasis, hepatocellular carcinoma (HCC), fibrolamellar HCC, undifferentiated embryonal sarcoma, cholangiocarcinoma, and angiosarcoma. HCC and hepatoblastoma are very unlikely the absence of underlying liver disease and considering patient’s age, respectively. Furthermore, progressive contrast enhancement on delayed images rather than early arterial enhancement argues against HCC. Undifferentiated embryonal sarcoma is another differential consideration that usually presents in the right hepatic lobe and is large at the time of presentation, similar to this case. However, this tumor is rare in patients older than 15 years, and on imaging, the lack of a cystic component and T2 bright matrix do not support this diagnosis. The lack of robust internal vascularity argues against angiosarcoma. Delayed progressive enhancement raises concern for cholangiocarcinoma, but in the absence of predisposing underlying disease such as choledochal cyst, primary sclerosing cholangitis, or inflammatory bowel disease, cholangiocarcinoma is very unlikely. Moreover, cholangiocarcinoma is usually T2 hyperintense [ 14 ]. CONCLUSION Pediatric SEF in the liver is extremely rare, and imaging findings are not described in the current literature. In the present case, significant posterior acoustic shadowing of the hypoechoic component of the hepatic mass on ultrasonography corresponding to a low-signal T2 appearance and progressive delayed enhancement on MRI are imaging clues that can help radiologists differentiate this tumor from other hepatic malignancies. References Warmke LMMD, Meis JMMD (2021) Sclerosing Epithelioid Fibrosarcoma: A Distinct Sarcoma with Aggressive Features. The American Journal of Surgical Pathology 45(3): p 317–328, March | 10.1097/PAS.0000000000001559 Woods AD, Purohit R, Mitchell LC et al (2021) Metastatic pediatric sclerosing epithelioid fibrosarcoma. Cold Spring Harbor Molecular Case Studies. ;7(5): a006093. DOI: 10.1101/mcs. a006093. PMID: 34362827; PMCID: PMC8559621 Kilaikode S, Kuril S, Sedrak A, Sadanandan S (2013) Sclerosing Epithelioid Fibrosarcoma of the Parietal Bone and Adjacent Meninges in an Adolescent: A Case Report. World J Oncol 4(6):255–257. 10.4021/wjon742w Epub 2014 Jan 16. PMID: 29147367; PMCID: PMC5649852 Zhang M, Yu Y, Guan X, Yao X, Jia C, Hong E, Guo Y, He L (2022) A group of sclerosing epithelioid fibrosarcomas with low-level amplified EWSR1-CREB3L1 fusion gene in children. Pathol Res Pract 230. https://doi.org/10.1016/j.prp.2021.153754 Armstrong A, Boulos F, Kulkarni S, Stoll J, Doyle MBM, Khan A, He M (2023 Mar-Apr) Sclerosing Epithelioid Fibrosarcoma of the Liver in a Pediatric Patient. Pediatr Dev Pathol 26(2):153–160 Epub 2023 Feb 6. PMID: 36748108 Moser T, Nogueira TS, Neuville A, Riehm S, Averous G, Weber J-C, Veillon F et al (2005) (1578–1580) Abdominal Imaging Moser. Fibrous Tumor of the Liver Case Report Delayed Enhancement Pattern in a Localized Fibrous Tumor of the Liver, American Journal of Roentgenology 184 https://doi.org/10.2214/ajr.184.5.01841578 Xia W, Yang Y, Huang Y (2020) Imaging Features of Sclerosing Epithelioid Fibrosarcoma of the Pancreas: A Case Report. Front Oncol 10. https://doi.org/10.3389/fonc.2020.00901 Goel N, Aryan S, Sivaraju L, Ghosal N (2017) Sclerosing epithelioid fibrosarcoma with brain metastases. Clin Neuropathol 36:86–89. https://doi.org/10.5414/NP300975 Shankar DS, Phan N, Shirsat H, Westra WH, Trosman SJ, Colley PM (2020) A 27-year-old female with sclerosing epithelioid fibrosarcoma of the left ethmoid sinus. Otolaryngol Case Rep 16. https://doi.org/10.1016/j.xocr.2020.100188 Rizk RC, Yasrab M, Chu LC, Weisberg EM, Fishman EK (2024) Metastatic sclerosing epithelioid fibrosarcoma. Radiol Case Rep 19:1815–1818. https://doi.org/10.1016/j.radcr.2024.01.080 Christensen DR, Ramsamooj R, Gilbert TJ, Gilbert TJ, Christensen DR, Ramsamooj R (1997) Sclerosing epithelioid fibrosarcoma: short T2 on MR imaging. Skeletal Radiol 26:619–621 Tomimaru Y, Nagano H, Marubashi S, Kobayashi S, Eguchi H, Takeda Y, Tanemura M, Kitagawa T, Umeshita K, Hashimoto N, Yoshikawa H, Wakasa K, Doki Y, Mori M (2009) Sclerosing epithelioid fibrosarcoma of the liver infiltrating the inferior vena cava. World J Gastroenterol 15:4204–4208. https://doi.org/10.3748/wjg.15.4204 Ding Q, Shao X, Liu X, Fu Y, Huang F, Wang C (2020) Case Report: Radiological Features of Sclerosing Epithelioid Fibrosarcoma in the Right Fibula, Front Oncol 10 November https://doi.org/10.3389/fonc.2020.603127 Dong Y, Cekuolis A, Schreiber-Dietrich D, Augustiniene R, Schwarz S, Möller K, Nourkami-Tutdibi N, Chen S, Cao JY, Huang YL, Wang Y, Taut H, Grevelding L, Dietrich CF (2023) Review on Pediatric Malignant Focal Liver Lesions with Imaging Evaluation: Part II. Diagnostics (Basel) 13(24):3659. 10.3390/diagnostics13243659 PMID: 38132242; PMCID: PMC10743166 Additional Declarations The authors declare no competing interests. Cite Share Download PDF Status: Posted Version 1 posted 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. Our growing team is made up of researchers and industry professionals working together to solve the most critical problems facing scientific publishing. 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-4694052","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":323322515,"identity":"66f3ad57-80bf-40a3-b9e4-4c10b897aa89","order_by":0,"name":"Ghazal Shadmani","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAAwklEQVRIiWNgGAWjYDACCRBRYAMiGQ8wMDATq8UgjQGkmiQth0nQIh/d/PjDD4PziRvOnz9wgKHCOrGBkBbDO8cMDHsMbiduuJEMtOVMOhFaZiQYJPAY3M7dcAPoMMa2w8RoSf9w8I/BudwN5w8DtfwjQou8RI5hM4/BgdwNB4AOY2wgQouBRE4xs4xBcv3MG8kGBxKOpRsTtmVG+uaPbyrsjPnOH3z44EONtSxhWw4g8xIIKQfbQtDQUTAKRsEoGAUAhopF4ONMOVEAAAAASUVORK5CYII=","orcid":"https://orcid.org/0000-0001-5274-4990","institution":"Washington university in Saint louis","correspondingAuthor":true,"prefix":"","firstName":"Ghazal","middleName":"","lastName":"Shadmani","suffix":""}],"badges":[],"createdAt":"2024-07-05 19:57:09","currentVersionCode":1,"declarations":{"humanSubjects":true,"vertebrateSubjects":false,"conflictsOfInterestStatement":false,"humanSubjectEthicalGuidelines":true,"humanSubjectConsent":true,"humanSubjectClinicalTrial":false,"humanSubjectCaseReport":true,"vertebrateSubjectEthicalGuidelines":false},"doi":"10.21203/rs.3.rs-4694052/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-4694052/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":59964242,"identity":"326e55b9-cdb3-49d4-8592-0ac47f4972b4","added_by":"auto","created_at":"2024-07-10 01:46:23","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":460795,"visible":true,"origin":"","legend":"\u003cp\u003e(A) Grayscale US shows a large hepatic mass with predominantly hypoechoic content and significant posterior acoustic shadowing (arrowheads). (B) Color Doppler US shows scanty peripheral vascularity with no significant internal vascularity.\u003c/p\u003e","description":"","filename":"1.png","url":"https://assets-eu.researchsquare.com/files/rs-4694052/v1/d53e85b87b403ca960897377.png"},{"id":59964244,"identity":"9e382d92-2afd-4670-880a-362913111596","added_by":"auto","created_at":"2024-07-10 01:46:23","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":1200787,"visible":true,"origin":"","legend":"\u003cp\u003e(A, B) Axial and coronal views of T2 weighted images (WI) show predominantly hypointense large hepatic mass. Note intrahepatic mild biliary dilation distal to the large mass (black arrows), splenomegaly, and multiple signal void collateral vessels (white arrows). In-phase (C) and out-phase (D) images do not show signal drop on out-phase image to suggest fat component. Diffusion weighted image (E) and Apparent Diffusion Coefficient (F) show peripheral restricted diffusion (white arrows) and mild T2 shine- through centrally.\u003c/p\u003e","description":"","filename":"2.png","url":"https://assets-eu.researchsquare.com/files/rs-4694052/v1/d4dc4cc05f7e039b789f86a1.png"},{"id":59964885,"identity":"e36be06f-0d56-417c-9286-23fbad8ead75","added_by":"auto","created_at":"2024-07-10 01:54:23","extension":"png","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":718514,"visible":true,"origin":"","legend":"\u003cp\u003eAxial T1 WI without contrast (A) and T1 WI immediate post contrast (B) show lack of early arterial enhancement. Axial T1WI 5 minutes post contrast (C) shows foci of enhancement which become more confluent and pronounced on axialT1WI 20 minutes post contrast (D).\u003c/p\u003e","description":"","filename":"3.png","url":"https://assets-eu.researchsquare.com/files/rs-4694052/v1/03e5d0b65b5d205e8006e195.png"},{"id":59964245,"identity":"d25f9fe7-2dd0-487e-a128-0a5da2bbcb77","added_by":"auto","created_at":"2024-07-10 01:46:23","extension":"png","order_by":4,"title":"Figure 4","display":"","copyAsset":false,"role":"figure","size":341672,"visible":true,"origin":"","legend":"\u003cp\u003eMaximum intensity projection (MIP) of FDG PET (A) shows mild to moderate uptake within the hepatic mass (blue arrows) and otherwise normal biodistribution of the tracer throughout the body with no PET findings of distant metastasis. Fused axial images of FGD PET and CT scan (B) show the most FDG avid areas of the mass. Note a focus of calcification in the mass on the non-contrast CT scan image (black arrow).\u003c/p\u003e","description":"","filename":"4.png","url":"https://assets-eu.researchsquare.com/files/rs-4694052/v1/55ab0939e77babe989ed17d0.png"},{"id":59964954,"identity":"52a7ad67-ba6e-463c-8e49-da3ae6c319f6","added_by":"auto","created_at":"2024-07-10 01:54:30","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":4133121,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-4694052/v1/82ef0c9b-59bd-4289-be31-bd90aa8c19e7.pdf"}],"financialInterests":"The authors declare no competing interests.","formattedTitle":"\u003cp\u003eImaging features of pediatric hepatic sclerosing epithelial fibrosarcoma\u003c/p\u003e","fulltext":[{"header":"INTRODUCTION","content":"\u003cp\u003eSclerosing epithelioid fibrosarcoma (SEF) is a rare variant of fibrosarcoma that shows round or oval epithelioid cells within a dense collagenous matrix. Although the histologic appearance of this tumor is benign with either an absent or low mitotic rate, the tendency toward local recurrence and synchronous/metachronous distant metastasis can reach 37% and 80%, respectively, suggesting that it is a clinically aggressive tumor [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e]. SFE mainly occurs in middle-aged adults without a significant sex predilection, with a reported median tumor size at presentation of approximately 8 cm. It is most commonly observed in the soft tissues of the lower extremity, trunk, upper extremity, and head and neck region. The primary sites of metastasis are the lung and bone [\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eFewer than a handful of pediatric cases have been described in the literature. The locations of SFE in pediatric patients presented in the literature include the bone[\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e], trunk (mediastinum, retroperitoneum, back, abdominal wall)[\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e], kidney[\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e], and liver[\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e]. Diagnosis is challenging due to the rarity of the disease, which is ultimately confirmed by pathology. Although the immunohistochemical and molecular genetic characteristics of pediatric hepatic SEF have been explored previously, no studies have focused on its imaging features. Therefore, the imaging characteristics of SEF remain poorly defined in the literature. The aim of this paper was to review the US, MRI, and FDG PET features of this rare pediatric liver malignancy. To the best of our knowledge, this is the first description of the multimodality imaging features of pediatric liver SEFs.\u003c/p\u003e"},{"header":"CASE REPORT","content":"\u003cp\u003eThe patient, which will be discussed, was evaluated at our center, and her parents consented to her case being reported in a medical journal.\u003c/p\u003e \u003cp\u003eA 17-year-old female, previously healthy, presented with abdominal pain and pruritus for several months. She had noticed jaundice in her eyes for the last two weeks but denied acholic stools. These symptoms were associated with 10-pound weight loss and fatigue. She was not on any steroids or oral contraceptive pills. Initial laboratory data revealed a total bilirubin level of 4.7 mg/dL (0.0-1.2), alkaline phosphatase level of 390 unit/L (40\u0026ndash;130), AST level of 101unit/L (10\u0026ndash;45), and ALT level of 162 unit/L (7\u0026ndash;45). Abdominal ultrasound (Fig.\u0026nbsp;1) revealed a large lobulated mixed echogenic mass centered on the right hepatic lobe containing several hypoechoic components with posterior acoustic shadowing without a discernible central scar. Color Doppler showed scant peripheral vascularity without significant internal vascularity. The spleen was moderately to markedly enlarged, with a craniocaudal dimension of approximately 19 cm. Upon further laboratory evaluation, CA 19\u0026thinsp;\u0026minus;\u0026thinsp;9 was 68.8 units/mL (Nl: up to 35), and her serum alpha-fetoprotein and beta-HCG levels were normal. Abdominal MRI (Figs.\u0026nbsp;2\u0026ndash;3) revealed a large predominantly hypointense mass on both T1- and T2-weighted images with heterogeneous enhancement on delayed images. There was no signal drop throughout the lesion on out-of-phase images to suggest fat component. No central scar was observed within the hepatic lesion. Mild, regular dilation of peripheral intrahepatic biliary ducts was also identified due to the mass effect on the biliary system. Splenomegaly and collateral vessels were present. There was no tumoral invasion or thrombosis of the inferior vena cava.\u003c/p\u003e \u003cp\u003eLiver biopsy confirmed the diagnosis of SEF. An FDG PET CT scan for initial staging showed mild to moderate uptake throughout the hepatic mass without evidence of distant metastatic disease (Fig.\u0026nbsp;4). Two months after the initial presentation, the patient subsequently underwent an orthotopic liver transplant. On the most recent surveillance imaging, 24 months after transplantation, no findings of disease recurrence were identified, and the serum CA 19\u0026thinsp;\u0026minus;\u0026thinsp;9 remained normal.\u003c/p\u003e"},{"header":"DISCUSSION","content":"\u003cp\u003eT2 hypointense appearance and progressive delayed contrast enhancement on MRI are well-known imaging features of fibrous tumors secondary to the high collagenous content of the tumor[\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e], both of which are present in this case. Another imaging clue suggesting the dense fibrous nature of the tumor is posterior acoustic shadowing of the hypoechoic components on US. Xia et al. described the imaging features of SFE in an adult patient with pancreatic SFE as T1/T2 hypointense pancreatic mass with progressive centripetal enhancement on delayed images [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]. Several prior case reports described the T2 hypointense appearance of intracranial metastases, ethmoid sinus, and intramuscular SEF [\u003cspan additionalcitationids=\"CR9 CR10\" citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e]. Tomimaru et al. presented the first case of SEF in a 35-year-old patient with a 7 cm right hepatic lobe mass and inferior vena cava invasion at the time of presentation with delayed centripetal enhancement on contrast-enhanced CT images [\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e]. This finding is expected considering the high fibrous content. Ding et al. described MRI findings in a 19-year-old patient with SEF of the right fibula as a T1/T2 hypointense mass with faint peripheral contrast enhancement during the arterial phase and progressive enhancement during the venous phase [\u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eSEF has been described as having a very slow growth process based on the observation of superficial soft tissue tumors. In the present case, the presence of cavernous venous formation at the porta hepatis and splenomegaly supported the slow growth of the SFE.\u003c/p\u003e \u003cp\u003eThe differential consideration in the present case included benign and malignant causes; benign lesions included hepatic adenoma and focal nodular hyperplasia. There was no signal drop on out-of-phase images for an internal fat component. Moreover, no T1 hyperintense foci of hemorrhage or central scarring were identified on T1-weighted images. The malignant differential considerations in this patient included metastasis, hepatocellular carcinoma (HCC), fibrolamellar HCC, undifferentiated embryonal sarcoma, cholangiocarcinoma, and angiosarcoma. HCC and hepatoblastoma are very unlikely the absence of underlying liver disease and considering patient\u0026rsquo;s age, respectively. Furthermore, progressive contrast enhancement on delayed images rather than early arterial enhancement argues against HCC.\u003c/p\u003e \u003cp\u003eUndifferentiated embryonal sarcoma is another differential consideration that usually presents in the right hepatic lobe and is large at the time of presentation, similar to this case. However, this tumor is rare in patients older than 15 years, and on imaging, the lack of a cystic component and T2 bright matrix do not support this diagnosis. The lack of robust internal vascularity argues against angiosarcoma. Delayed progressive enhancement raises concern for cholangiocarcinoma, but in the absence of predisposing underlying disease such as choledochal cyst, primary sclerosing cholangitis, or inflammatory bowel disease, cholangiocarcinoma is very unlikely. Moreover, cholangiocarcinoma is usually T2 hyperintense [\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e].\u003c/p\u003e"},{"header":"CONCLUSION","content":"\u003cp\u003ePediatric SEF in the liver is extremely rare, and imaging findings are not described in the current literature. In the present case, significant posterior acoustic shadowing of the hypoechoic component of the hepatic mass on ultrasonography corresponding to a low-signal T2 appearance and progressive delayed enhancement on MRI are imaging clues that can help radiologists differentiate this tumor from other hepatic malignancies.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\u003cli\u003e\u003cspan\u003eWarmke LMMD, Meis JMMD (2021) Sclerosing Epithelioid Fibrosarcoma: A Distinct Sarcoma with Aggressive Features. The American Journal of Surgical Pathology 45(3): p 317\u0026ndash;328, March | \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1097/PAS.0000000000001559\u003c/span\u003e\u003cspan address=\"10.1097/PAS.0000000000001559\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eWoods AD, Purohit R, Mitchell LC et al (2021) Metastatic pediatric sclerosing epithelioid fibrosarcoma. Cold Spring Harbor Molecular Case Studies. ;7(5): a006093. DOI: 10.1101/mcs. a006093. PMID: 34362827; PMCID: PMC8559621\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eKilaikode S, Kuril S, Sedrak A, Sadanandan S (2013) Sclerosing Epithelioid Fibrosarcoma of the Parietal Bone and Adjacent Meninges in an Adolescent: A Case Report. World J Oncol 4(6):255\u0026ndash;257. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.4021/wjon742w\u003c/span\u003e\u003cspan address=\"10.4021/wjon742w\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003eEpub 2014 Jan 16. PMID: 29147367; PMCID: PMC5649852\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eZhang M, Yu Y, Guan X, Yao X, Jia C, Hong E, Guo Y, He L (2022) A group of sclerosing epithelioid fibrosarcomas with low-level amplified EWSR1-CREB3L1 fusion gene in children. Pathol Res Pract 230. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1016/j.prp.2021.153754\u003c/span\u003e\u003cspan address=\"10.1016/j.prp.2021.153754\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eArmstrong A, Boulos F, Kulkarni S, Stoll J, Doyle MBM, Khan A, He M (2023 Mar-Apr) Sclerosing Epithelioid Fibrosarcoma of the Liver in a Pediatric Patient. Pediatr Dev Pathol 26(2):153\u0026ndash;160 Epub 2023 Feb 6. PMID: 36748108\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eMoser T, Nogueira TS, Neuville A, Riehm S, Averous G, Weber J-C, Veillon F et al (2005) (1578\u0026ndash;1580) Abdominal Imaging Moser. Fibrous Tumor of the Liver Case Report Delayed Enhancement Pattern in a Localized Fibrous Tumor of the Liver, American Journal of Roentgenology 184 \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.2214/ajr.184.5.01841578\u003c/span\u003e\u003cspan address=\"10.2214/ajr.184.5.01841578\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eXia W, Yang Y, Huang Y (2020) Imaging Features of Sclerosing Epithelioid Fibrosarcoma of the Pancreas: A Case Report. Front Oncol 10. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.3389/fonc.2020.00901\u003c/span\u003e\u003cspan address=\"10.3389/fonc.2020.00901\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eGoel N, Aryan S, Sivaraju L, Ghosal N (2017) Sclerosing epithelioid fibrosarcoma with brain metastases. Clin Neuropathol 36:86\u0026ndash;89. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.5414/NP300975\u003c/span\u003e\u003cspan address=\"10.5414/NP300975\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eShankar DS, Phan N, Shirsat H, Westra WH, Trosman SJ, Colley PM (2020) A 27-year-old female with sclerosing epithelioid fibrosarcoma of the left ethmoid sinus. Otolaryngol Case Rep 16. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1016/j.xocr.2020.100188\u003c/span\u003e\u003cspan address=\"10.1016/j.xocr.2020.100188\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eRizk RC, Yasrab M, Chu LC, Weisberg EM, Fishman EK (2024) Metastatic sclerosing epithelioid fibrosarcoma. Radiol Case Rep 19:1815\u0026ndash;1818. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1016/j.radcr.2024.01.080\u003c/span\u003e\u003cspan address=\"10.1016/j.radcr.2024.01.080\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eChristensen DR, Ramsamooj R, Gilbert TJ, Gilbert TJ, Christensen DR, Ramsamooj R (1997) Sclerosing epithelioid fibrosarcoma: short T2 on MR imaging. Skeletal Radiol 26:619\u0026ndash;621\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eTomimaru Y, Nagano H, Marubashi S, Kobayashi S, Eguchi H, Takeda Y, Tanemura M, Kitagawa T, Umeshita K, Hashimoto N, Yoshikawa H, Wakasa K, Doki Y, Mori M (2009) Sclerosing epithelioid fibrosarcoma of the liver infiltrating the inferior vena cava. World J Gastroenterol 15:4204\u0026ndash;4208. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.3748/wjg.15.4204\u003c/span\u003e\u003cspan address=\"10.3748/wjg.15.4204\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eDing Q, Shao X, Liu X, Fu Y, Huang F, Wang C (2020) Case Report: Radiological Features of Sclerosing Epithelioid Fibrosarcoma in the Right Fibula, Front Oncol 10 November \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.3389/fonc.2020.603127\u003c/span\u003e\u003cspan address=\"10.3389/fonc.2020.603127\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eDong Y, Cekuolis A, Schreiber-Dietrich D, Augustiniene R, Schwarz S, M\u0026ouml;ller K, Nourkami-Tutdibi N, Chen S, Cao JY, Huang YL, Wang Y, Taut H, Grevelding L, Dietrich CF (2023) Review on Pediatric Malignant Focal Liver Lesions with Imaging Evaluation: Part II. Diagnostics (Basel) 13(24):3659. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.3390/diagnostics13243659\u003c/span\u003e\u003cspan address=\"10.3390/diagnostics13243659\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003ePMID: 38132242; PMCID: PMC10743166\u003c/span\u003e\u003c/li\u003e\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":true,"hideJournal":true,"highlight":"","institution":"Washington University in St. Louis","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true},"keywords":"Sclerosing epithelioid fibrosarcoma (SEF), liver, pediatric ","lastPublishedDoi":"10.21203/rs.3.rs-4694052/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-4694052/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003e\u003cstrong\u003eBackground\u003c/strong\u003e: Sclerosing epithelioid fibrosarcoma (SEF) is a rare variant of fibrosarcoma that has been described in fewer than a handful of pediatric patients in the literature and in almost all prior publications focused on pathology. There is no description of the imaging features of hepatic SEF in the literature.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003ePatient presentation\u003c/strong\u003e: A 17-year-old female with chronic abdominal pain and pruritis was found to have a large solid mass in the right hepatic lobe on ultrasonography (US). Further imaging by contrast-enhanced MRI and FDG PET was performed. A core biopsy of the hepatic mass revealed SFE. Two months later, she underwent liver transplantation. On clinical and imaging follow-up up to 24 months after transplantation, no evidence of recurrence or metastasis was identified.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConclusion\u003c/strong\u003e:\u003cstrong\u003e \u003c/strong\u003eThis paper presents the first multimodality imaging features of primary hepatic SEF in an adolescent patient.\u003c/p\u003e","manuscriptTitle":"Imaging features of pediatric hepatic sclerosing epithelial fibrosarcoma","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2024-07-10 01:46:18","doi":"10.21203/rs.3.rs-4694052/v1","editorialEvents":[{"type":"communityComments","content":0}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"50f2e4d9-0807-469e-bf15-7aabda52cbf8","owner":[],"postedDate":"July 10th, 2024","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[{"id":34197425,"name":"Pediatrics"},{"id":34197426,"name":"Oncology"},{"id":34197427,"name":"Nuclear Medicine \u0026 Medical Imaging"}],"tags":[],"updatedAt":"2024-07-18T03:54:35+00:00","versionOfRecord":[],"versionCreatedAt":"2024-07-10 01:46:18","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-4694052","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-4694052","identity":"rs-4694052","version":["v1"]},"buildId":"qtupq5eGEP_6zYnWcrvyt","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}