Abstract
Epithelioid mesenchymal malignancies represent a major diagnostic challenge. Epithelioid morphology can be observed in a variety of soft tissue neoplasms, however there exist specific subtypes in which an epithelioid apperance constitutes the most distinctive morphological feature. Moving from epithelioid sarcoma of Enzinger (the prototype of sarcoma with epithelioid morphology), this review will focus on the most relevant entities: namely epithelioid haemangioendothelioma and angiosarcoma, pseudomyogenic haemangioendothelioma, epithelioid malignant peripheral nerve sheath tumour, epithelioid sclerosing fibrosarcoma, epithelioid pleomorphic liposarcoma, alveolar soft part sarcoma, and undifferentiated soft tissue sarcoma with epithelioid morphology. Differential diagnoses and major pitfalls will be discussed in detail.
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