The PECs also exhibit a distinct immunophenotype with a coexpression of melanocytic and myogenic markers, such as HMB45, Melan-A, MiTF, smooth muscle actin (SMA), and calponin.2 The PEComa family includes angiomyolipoma (AML), clear cell “sugar” tumor of the lung (CCST), lymphangioleiomyomatosis (LAM), clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres (CCMMT), and unusual clear cell tumors in other locations. This evidence concerns the gene SMN1 and lymphangioleiomyomatosis.