Mucocutaneous Angiomyolipoma of Premaxillary region Masquerading as Lipoma: a known but rare occurrence

Angiomyolipomas (AML) are distinctive benign mesenchymal tumors seen predominantly in the kidney. Majority (80%) of these tumors are sporadic, while remaining 20% of them are associated with tuberous sclerosis (TS). Extrarenal angiomyolipomas are extremely rare, liver being the commonest site followed by skin. Although rare, skin is the commonest reported site and literature search revealed cases of extrarenal angiomyolipomas arising at unusual locations like scrotal skin, vulva, colon and extrarenal peritoneum among others. We present a case of mucocutaneous angiomyolipoma located in left premaxillary region in a man aged 53years. Although head is the most commonly reported region for mucocutaneous AML, present case of premaxillary region angiomyolipoma presents as an intriguing mass in a region which is the best example for mucocutaneous site as the mass was located in-between skin and buccal mucosa. Lesion was excised and subsequent histopathological examination revealed the true nature of lesion. AML should be included in the differential diagnosis of premaxillary soft tissue masses.