Update on perivascular epithelioid cell tumors of the urinary system / 国际外科学杂志

ABSTRACT

The perivascular epithelioid cell (PEC) is a cell type constantly present in a group of tumors called PEComas(perivascular epithelioid cell tumors). PEC expresses myogenic and melanocytic markers,such as HMB45. PEComa is a widely accepted entity now. PEComas are related to the genetic alterations of tuberous sclerosis complex (TSC), an autosomal dominant genetic disease due to losses of TSC1 or TSC2 genes. PEComas are rare in the urinary system and there are some open questions about PEComas regarding its histogenesis, the definition of epithelioid angiomyolipoma and the identification of the histological criteria of malignancy. This review provides an update on PEComas of the urinary system.