Management of cutaneous angiomyolipoma and its association with tuberous sclerosis

Abstract

Although typically presenting renally, angiomyolipomas can rarely present in the skin. The tumors are composed of an admixture of blood vessels, smooth muscle and adipose tissue and are often seen in the setting of tuberous sclerosis. We describe a case of angiomyolipoma presenting on the thigh of a 50-year-old white female. This is the 17th reported case of cutaneous angiomyolipoma. As with all previously described cases, our patient did not present with the stigmata of tuberous sclerosis. Angiomyolipoma should be considered within the differential for subcutaneous nodules and work-up for tuberous sclerosis should not be pursued when presenting in the skin.

Case of a cutaneous angiomyolipoma in the ear

“…Cutaneous angiomyolipoma presents as an asymptomatic, subcutaneous tumor. Reported locations have primarily been acral regions, including the ear, nose, fingers, toes and elbows 8–10 . Among 18 reported cases, including the present case, nine occurred in the ear and periauricular region.…”

“…Renal angiomyolipoma is associated with tuberous sclerosis in one‐third of cases 1 . On the other hand, no cases of cutaneous angiomyolipoma have been associated with tuberous sclerosis to date 2–10 . In this case, the patient had no signs of tuberous sclerosis.…”

“…Histopathologically, angiomyolipoma is composed of mature adipose tissue, blood vessels and interdigitating smooth muscle cell bundles 1 . Cutaneous angiomyolipoma is a rare type of tumor, and most commonly occurs on the ear or in the acral skin 2–10 . Here, we report a case of a cutaneous angiomyolipoma on the ear lobule.…”

“…Unlike renal angiomyolipoma, which is positive for HMB‐45, cutaneous angiomyolipoma shows no such reactivity 5,7–10 . In addition, the renal type shows female predominance, whereas the cutaneous type shows male predominance 2,7–10 . Based on these differences, cutaneous angiomyolipoma should be considered as a different disease to the renal type.…”

Facial angiofibromas of tuberous sclerosis: successful treatment with podophyllin

“…The treatment of facial angiofibromas has been a problem because of the multiplicity of the lesions and the presence of the lesions at anatomically difficult sites such as nasolabial folds, chin, upper lip, nose, and periorbital areas. Several treatment modalities causing non-selective destruction of tissue such as curettage, 3 dermabrasion, 4 chemical peeling, 3 cryotherapy, 5 electrosurgery, 6 excision, 4 and lasers [7][8][9] have been used for the treatment with…”