The same group coined the term “perivascular epithelioid cell” (PEC) for these cells in 1992 and proposed the presence of this distinctive cell, distinguished in part by strong HMB-45 positivity, as a common link between a number of rare disorders in disparate locations, including AML, CCST, and lymphangiomyomatosis (LAM) of the lung [3]. This evidence concerns the gene PMEL and lymphangioleiomyomatosis.