Congenital superficial angiomyxoma

Summary Superficial angiomyxomas are rare, benign, dermal and subcutaneous tumours. We describe a 12‐year‐old girl who presented with a nodular swelling in the midline of her scalp that had been present since birth. Histological examination revealed an ill‐defined myxoid lesion within the dermis, comprising spindle cells, blood vessels and occasional multi‐nucleate giant cells. Immunohistochemical staining was negative for S‐100, cytokeralin and smooth muscle actin, hut locally positive for CD34, Our patient is unusual in that the angionnxoma was present at birth, which has not previously been described. The importance of screening patients with cutaneous myxomas for cardiac lesions is discussed. myxomas were described in 1948 by Stout as ‘true neoplasms composed of stellate cells set in a loose mucoid stroma through which course very delicate reticulin fibres in various directions' and ‘resemble primitive mescnchyme’. 1 They are found throughout the body, most commonly in skeletal muscle and heart, but also in the subcutaneous tissue, pelvis and bone. The cutaneous myxoma is rare. Those lesions with a predominant vascular component are termed ‘angiomyxomas’. They are usually solitary, potentially locally recurrent but non‐metastatic, and occur most commonly in the fourth decade. To date, the youngest patient reported with a cutaneous angiomyxoma was 2 years old.