NDLI: Sclerosing epithelioid fibrosarcoma as a rare cause of ascites in a young man: a case report

Content Provider	Springer Nature : BioMed Central
Author	Smith, Philip J Almeida, Beverley Krajacevic, Jasna Taylor, Barry
Abstract	Introduction Sclerosing epithelioid fibrosarcoma is a rare but distinct variant of fibrosarcoma that not only presents as a deep-seated mass on the limbs and neck but can also occur adjacent to the fascia or peritoneum, as well as the trunk and spine. We report the case of an intra-abdominal sclerosing epithelioid fibrosarcoma, which to best of the authors' knowledge has not been described previously. The patient discussed here developed lung metastases but is still alive 1-year post-diagnosis. Case presentation A 29-year-old man presented with a 2-week history of progressive abdominal distension and pain and was found to have marked ascites. A full liver screen was unremarkable with abdominal and chest computed tomography scans only confirming ascites. After a diagnostic laparotomy, biopsies were taken from the greater omentum and peritoneal nodules. Histopathology revealed a malignant tumour composed of sheets and cords of small round cells set in collagenized stroma. After further molecular investigation at the Mayo Clinic, USA, the diagnosis of a high-grade sclerosing epithelioid fibrosarcoma was confirmed. Conclusion Sclerosing epithelioid fibrosarcoma is an extremely rare tumour, which is often difficult to diagnose and which few pathologists have encountered. This case is particularly unusual because of the intra-abdominal origin of the tumour. Owing to the rarity of sclerosing epithelioid fibrosarcoma, there is no clear evidence regarding the prognosis of such a tumour, although sclerosing epithelioid fibrosarcoma is able to metastasize many years post-presentation. It is important that physicians and pathologists are aware of this unusual tumour.
Related Links	https://jmedicalcasereports.biomedcentral.com/counter/pdf/10.1186/1752-1947-2-248.pdf
Ending Page	3
Page Count	3
Starting Page	1
File Format	HTM / HTML
ISSN	17521947
DOI	10.1186/1752-1947-2-248
Journal	Journal of Medical Case Reports
Issue Number	1
Volume Number	2
Language	English
Publisher	BioMed Central
Publisher Date	2008-07-25
Access Restriction	Open
Subject Keyword	Medicine Public Health General Practice Family Medicine Primary Care Medicine Surgical Oncology Internal Medicine Synovial Sarcoma Small Round Cell Round Cell Tumour Clear Cell Sarcoma Desmoplastic Small Round Cell Tumour Medicine/Public Health
Content Type	Text
Resource Type	Case study
Subject	Medicine
Journal Impact Factor	0.9/2023

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