Cutaneous angiomyolipomas are HMB45 negative, not associated with tuberous sclerosis, and should be considered as angioleiomyomas with fat.

Angiomyolipomas are uncommon tumors in the skin. Neoplasms with the same name are more typically associated with a renal location, many in the context of tuberous sclerosis. Three cases of cutaneous angiomyolipoma are reported, which were all HMB45 negative and not associated with tuberous sclerosis. This is the first series of such tumors to be stained with HMB45. The presence of fat in histologically similar cutaneous angioleiomyomas has been previously documented and a review of 40 archival cases revealed fat in 1 case. The author proposes that cutaneous angiomyolipomas should be termed angioleiomyoma with fat to avoid confusion with unrelated non-cutaneous angiomyolipomas that are HMB45 negative and require investigation for tuberous sclerosis.