perivascular cells |
perivascular fibroblasts |
spheroid-forming unit |
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[perivascular epithelioid cell]
This cell type is present in a group of tumors (often referred to as PEComas), which includes angiomyolipoma, clear-cell "sugar" tumor of the lung and extrapulmonary sites, lymphangioleiomyomatosis (see also: LAM cells), clear-cell myomelanocytic tumor of the falciform ligament/ligamentum teres and rare clear-cell tumors of other anatomical sites. These tumors are related to the genetic alterations of tuberous sclerosis complex (TSC), an autosomal dominant genetic disease due to losses of TSC1 (9q34) or TSC2 (16p13.3) genes. (Martignoni et al, 2008; Folpe and Kwiatkowski, 2010).
The cells have distinctive morphologic, immunohistochemical, ultrastructural and genetic features. The cells are known to express various myogenic and melanocytic
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