Epithelioid Soft Tissue Tumors: A Clinicopathologic and Molecular Update

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Abstract

Soft tissue tumors with epithelioid morphology are challenging to diagnose. These rare neoplasms can closely mimic more common tumor types, especially carcinomas and melanoma. In this review, we discuss the clinicopathologic and immunohistochemical features of select epithelioid soft tissue tumors, focusing on those for which the molecular pathogenesis has recently been clarified: epithelioid inflammatory myofibroblastic sarcoma (with RANBP2-ALK or RRBP1-ALK fusions), sclerosing epithelioid fibrosarcoma (with EWSR1-CREB3L1 or FUS-CREB3L2 fusions), ossifying fibromyxoid tumor (most often with PHF1 rearrangement), epithelioid malignant peripheral nerve sheath tumor (with SMARCB1 inactivation), epithelioid hemangioma (with FOSB or FOS fusions), epithelioid hemangioendothelioma (with WWTR1-CAMTA1 or YAP1-TFE3 fusions), and epithelioid angiosarcoma (rarely with CIC alterations). Finally, for each of these entities, we review approaches to differential diagnosis.

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