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Case Reports

Abstract

Cutaneous angiosarcoma is a malignant vascular neoplasm which arises in chronically sun-damaged skin, or secondarily following therapeutic radiation or in long-standing lymphedema. A wide range of histopathological features of the malignant cells have been reported, however, overlying epidermal hyperplastic changes are rarely described. Here we present a case of secondary cutaneous angiosarcoma in a 58-year-old man who presented with pain and swelling overlying the radiation site of a previously treated MDM2-amplified liposarcoma of the distal thigh. Physical examination demonstrated a nodular, tan-pink, verrucous lesion extending from the lower thigh to the dorsal foot with circumferential extension around the calf (39.8 x 35.1 cm). Biopsy of soft tissue overlying the right femur showed angiosarcoma and a palliative above-the-knee amputation was performed. The resection confirmed a high-grade angiosarcoma with microscopic findings of plump, pleomorphic, epithelioid endothelial tumor cells invading the dermis and underlying structures. Prominent epidermal changes associated with the clinically described plaque overlayed the dermal vascular tumor, characterized by verrucous epithelial hyperplasia with papillomatosis, compact orthokeratosis, and hypergranulosis. Together, these findings are consistent with verrucous angiosarcoma of the skin. Including this case, only 5 have been reported in the literature. Interestingly, all are associated with an epithelioid morphology, which confers a worse prognosis. All were secondary to either radiation or chronic lymphedema and occurred in the extremities. The pathophysiologic mechanisms which give rise to this verrucous presentation are unknown. Recognition of these features may aid in the diagnosis of angiosarcoma, which is not usually considered in differential of papillomatous lesions.