PEComas: Neoplasms with perivascular epithelioid cell differentiation

PEComas, occasionally associated with tuberous sclerosis complex, are defined by the presence of perivascular epithelioid cells that coexpress muscle and melanocytic markers. The family of tumors includes angiomyolipoma, clear cell sugar tumor of the lung, limphangioleiomyomatosis and very rare tumors in the other locations, which natural history and prognostic features undefined. The majority of PEComas are benign. Clear criteria for malignancy in PEComas have not be elaborated, owning to their rarity. Histogenesis of PEComas is unknown.

PEComa, classification, histopathology, histogenesis, clinical features