Perivascular Epithelioid Cell Tumors (PEComa) – Market Outlook, Epidemiology, Competitive Landscape, and Market Forecast Report – 2023 To 2033

Perivascular epithelioid cell tumors (PEComa) are a family of rare mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. These tumors include renal angiomyolipoma; lymphangioleiomyomatosis; clear cell sugar tumor of the lung; and similar tumors occurring in the retroperitoneum, kidney, uterus, and pancreas and in a variety of other visceral and soft-tissue sites. PEComas of the kidney includes various histologic subtypes of angiomyolipomas such as the classic angiomyolipoma, epithelioid angiomyolipoma, oncocytomalike angiomyolipoma, and lymphangiomyomatosis of the renal sinus. PEComas are related to the tuberous sclerosis complex (TSC) and have the characteristic genetic alterations of TSC including the deletions of the short arm of chromosome 16p (TSC2 gene). PEComas are histologically characterized by their perivascular location, usually epithelioid or occasionally spindle cell morphology, and coexpression of human me...