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/Objective Cutaneous angiomyolipomas (AML) are rare lesions that have morphological similarities to renal AML, but appear to be biologically distinct. Methods/Case Report We report the case of a 69-year-old man who presented with a slowly enlarging nodule on the dorsum of the right hand that was painful upon wrist flexion and is slightly tender to palpation. A ganglion cyst was clinically suspected, and the lesion was excised. Microscopically, this was a well-circumscribed nodule with a thin fibrous capsule that was highlighted by a trichrome stain. The lesion was composed of thick-walled blood vessels surrounded by bundles of smooth muscles that intermingled with adipose tissue. Smooth muscle actin immunostain highlighted the smooth muscles, but HMB-45 was negative. There was no atypia, and no mitoses. A diagnosis of “cutaneous angiomyolipoma” was made. Results (if a Case Study enter NA) NA Conclusion A review of the limited number of published cases showed that unlike renal AML, this lesion is not associated with tuberous sclerosis, is negative for melanocytic markers, and shows male-predominance, suggesting that the two morphologically similar lesions are biologically unrelated. The 2018 WHO classification of skin Tumors does not list this entity, so nomenclature is not standardized. Some authors suggested that these are simply angioleiomyomas with prominent adipose tissue, but the female predominance of angioleiomyoma makes this unlikely. Others have recognized this as a distinct entity and described it as an “angiolipoleiomyoma”, to distinguish it from renal AML. We now advocate for the latter approach to avoid confusion with renal AML. Otherwise, the prefix of “cutaneous” should always be used in the diagnosis to highlight this distinction.

Cutaneous Angiomyolipoma (Angiolipoleiomyoma): Case Report and Review of Literature with Emphasis on Nomenclature