Reed syndrome presenting with leiomyosarcoma

FH: Fumarate hydratase HLRCC: Hereditary leiomyomatosis and renal cell cancer INTRODUCTION Hereditary leiomyomatosis and renal cell cancer (HLRCC), also known as familial leiomyomatosis cutis et uteri or Reed syndrome, is a rare autosomal dominant disorder that manifests primarily as skin and uterine leiomyomas, usually at earlier ages than in the general population. Renal cell carcinoma also develops in a subset of affected individuals. Cutaneous and subcutaneous leiomyomas are rare benign smooth-muscle tumors that can arise from the arrector pili muscle, dartos muscles in genital skin, or vascular smooth muscle. Cutaneous leiomyosarcomas account for 2% to 3% of all soft tissue sarcomas and have been reported infrequently in patients with Reed syndrome. We report the case of a 44-year-old white woman with Reed syndrome who presented with a primary cutaneous leiomyosarcoma in the right postauricular region, emphasizing the importance of cutaneous surveillance in patients affected by Reed syndrome.