Leiomyoma cutis: a focused review on presentation, management, and association with malignancy

Cutaneous leiomyomas (CLs) are rare, sporadic, or inherited tumors of smooth muscle origin associated with various disorders. Hereditary leiomyomatosis and renal cell cancer (HLRCC) is the primary tumor predisposition syndrome associated with inherited CLs, affecting 180 families worldwide, with significant mortality. CLs are subdivided into piloleiomyomas, genital leiomyomas, and angioleiomyomas based on their smooth muscle of origin, as well as their clinicopathologic features. Piloleiomyomas, derived from arrector pili muscle, are solitary or multiple firm papulonodules located typically on the extremities and trunk; genital leiomyomas, derived from dartoic, vulvar, or mammary smooth muscle, are solitary papulonodules or pedunculated papules located on the scrotum, vulva, or nipple; and angioleiomyomas, which include solid, cavernous, or venous subtypes, are derived from the tunica media of small arteries and veins and typically present on the extremities. Partial/excisional biopsy is required for diagnosing all CLs. Histology shows interlacing fascicles of spindle cells with moderate amounts of eosinophilic cytoplasm and a blunt-ended, elongated nucleus with perinuclear halos. Surgical excision is curative for CLs, with other management options including medical or destructive therapy; active surveillance is advised to monitor HLRCC-associated neoplasms, with pharmacological therapies under active research.