Update on perivascular epithelioid cell tumors of the urinary system
10.3760/cma.j.issn.1673-4203.2010.08.016
- VernacularTitle:泌尿系统血管周围上皮样细胞瘤家族的研究现状
- Author:
Daoxin ZHANG
;
Wenying WANG
;
Lindong DU
;
Ye TIAN
- Publication Type:Journal Article
- Keywords:
Perivascular epithelioid cell tumors;
Perivascular epithelioid cell;
Urinary system
- From:
International Journal of Surgery
2010;37(8):551-554
- CountryChina
- Language:Chinese
-
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.
