SOFT TISSUE EPITHELIOID VASCULAR TUMORS: A PRACTICAL CLINICO PATHOLOGICAL DIAGNOSTIC APPROACH

Soft tissue epithelioid vascular tumors are characterized by a wide spectrum and represent a controversial topic for overlapping morphologic features; they require depth knowledge of the various subtypes, in order to be able to predict the clinical behaviour of the lesion. The World Health Organization (WHO) classification of soft tissue tumours recognizes multiple vascular tumours with epithelioid features, with different levels of malignancy: 1) benign neoplasms, as epithelioid hemangioma (EH) 2) tumours with an intermediate behaviour, characterized by low distant metastatic rates, including pseudomyogenic hemangioendothelioma (PHE); 3) malignant vascular tumours, with higher metastatic rate, as epithelioid hemangioendothelioma (EHE) and the epithelioid variant of angiosarcoma (EAS). The aim of this review is to report the most important clinical, pathological, immuhistochemical and cytogenetic features of epithelioid vascular tumours. In order to reach the correct diagnosis, the identification of epithelioid features, immunohistochemical (CD31 and ERG) and cytogenetic (CAMPTA1 and FLI1) markers are able to make more reproducible the interpretation of this complex group of soft tissue neoplasms.