Sclerosing epithelioid fibrosarcoma – a rare variant with myriads of differentials

Sclerosing Epithelioid Fibrosarcoma is a rare form of soft tissue sarcoma, with increased propensity for local recurrence and metastases mainly pulmonary and pleural in about 50% of cases. It carries morphological resemblance with many sarcomas, hence detailed and comprehensive immunohistochemical e...

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Bibliographic Details
Published in:Surgical and experimental pathology 2023-10, Vol.6 (1), p.1-3, Article 14
Main Authors: Prachi, Aiyer, Hema Malini
Format: Article
Language:English
Subjects:
MUC4 positivity
Rare malignant mesenchymal neoplasm
Sclerosing epithelioid fibrosarcoma
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Summary:Sclerosing Epithelioid Fibrosarcoma is a rare form of soft tissue sarcoma, with increased propensity for local recurrence and metastases mainly pulmonary and pleural in about 50% of cases. It carries morphological resemblance with many sarcomas, hence detailed and comprehensive immunohistochemical examination is mandatory for definitive diagnosis. Hereby, we present a case in a 39 year old male, who presented with a painless right thigh swelling. The histopathological examination revealed nests of epithelioid cells embedded in a densely sclerotic matrix, and immunohistochemistry showed MUC4 and Vimentin positivity, rendering the final diagnosis of Sclerosing Epithelioid Fibrosarcoma.
ISSN:2520-8454
2520-8454
DOI:10.1186/s42047-023-00139-9