Otherforms include lymphangioleiomyomatosis (LAM) and clear cell “sugar” tumor of thelung.4,5 Perivascular epithelioidcell tumors may contain epithelioid or spindle-shaped cells while their cytoplasm can rangefrom being clear to eosinophilic.4 Nearly all PEComas show immunoreactivity for both melanocytic (HMB-45 and/or melan-A)and smooth muscle markers. The gene discussed is MLANA; the disease is lymphangioleiomyomatosis.