Angiomyolipoma of the helix without signs of systemic involvement: successful surgical approach.

Cutaneous angiomyolipoma (AMP) of the ear is an extremely rare benign mesenchymal tumour. Angiomyolipomas are the most common benign tumour of the kidney and could be associated in 20% of the cases with the tuberous sclerosis complex (TBC), in which condition, most of the patients have several angiomyolipomas affecting both of the kidneys. We report the rare case of a 66-year-old female who had an asymptomatic, solitary, nodule on the helix of the right ear for several years, which was histologically assessed as angiomyolipoma. There were no clinical signs of the tuberous sclerosis complex (TBC) or renal AML. A surgical excision was performed with an excellent therapeutic result. In contrast to renal AMLs or PEComas, which are often invasive and may involve regional nodes, cutaneous AMLs are solitary, non-invasive, and not associated with tuberous sclerosis, and are curable by simple elliptical excision.