Angiomyolipoma, Mediastinal

Angiomyolipoma is a usually benign mesenchymal neoplasm composed of smooth muscle, blood vessels, and adipose tissue in varying amounts (Morita et al. 2012). It occurs predominantly in the kidney but can rarely occur in extrarenal locations including the liver, lungs, and the mediastinum (Candaș et al. 2013). Though they may be sporadic in origin, angiomyolipomas are associated with tuberous sclerosis and TSC2/PKD1 contiguous gene syndrome (Martignoni et al. 2002). Patients with a genetic syndrome tend to present at a younger age than patients without a genetic mutation. Angiomyolipomas belong to the perivascular epithelioid cell tumor (PEComa) family, which means they have perivascular and epithelioid features and can coexpress melanocytic and muscle markers. The PEComa family includes angiomyolipoma (AML), clear-cell sugar tumor (CCST) of the lung, lymphangioleiomyomatosis (LAM), clear-cell myomelanocytic tumor of the falciform ligament/ligamentum teres, and other rare clear-cell...