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[Epithelioid sarcoma: morphologic variants and differential diagnosis].

¹Dermatopathologie Bodensee, Siemensstr. 6/1, 88048, Friedrichshafen, Deutschland. mentzel@dermpath.de

|December 10, 2009

Summary

Epithelioid sarcoma is a rare cancer primarily affecting young adults, often misdiagnosed as benign. Most cases show loss of INI1, crucial for diagnosis and understanding this aggressive sarcoma.

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Induction of Mesenchymal-Epithelial Transitions in Sarcoma Cells

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Published on: April 7, 2017

Area of Science:

Oncology
Pathology
Molecular Biology

Context:

Epithelioid sarcoma is a rare soft tissue sarcoma with a poor prognosis.
It predominantly affects young adults on the distal extremities.
Often presents with features mimicking benign conditions, complicating early diagnosis.

Purpose:

To describe the histological and immunohistochemical characteristics of epithelioid sarcoma.
To highlight the significance of INI1 (SMARCB1) loss in epithelioid sarcoma diagnosis.
To differentiate epithelioid sarcoma from its variants and other neoplasms.

Summary:

Histologically, epithelioid sarcoma exhibits multinodular growth with central necrosis and uniform epithelioid cells.
Immunohistochemically, it shows coexpression of vimentin, epithelial membrane antigen, and pancytokeratin; CD34 is positive in about half of cases.
Loss of INI1 (SMARCB1) is a key feature in most epithelioid sarcoma cases, distinguishing it from other sarcomas.

Impact:

Understanding these features aids in accurate diagnosis and differential diagnosis of epithelioid sarcoma.
Recognition of variants like proximal, fibroma-like, angiomatoid, and myxoid is crucial.
The frequent loss of INI1 provides a potential diagnostic marker and insight into the pathogenesis of this rare sarcoma.