Angiosarcoma of the Face

September 14, 2005
Johnathan S. Crane, MD
Johnathan S. Crane, MD

,
Warren White, MD
Warren White, MD

,
Peter G. Kramer, MD
Peter G. Kramer, MD

The patient reported that he had noticed the facial eruption a couple of months earlier. On examination, the lesion appeared more plaque-like and dense than is typical of rosacea. The differential diagnosis included lupus, mycosis fungoides, rosacea, and angiosarcoma.

Following 3 weeks of unsuccessful treatment with metronidazole cream, this 71-year-old man was referred to our office for treatment of rosacea, write Drs Jonathan S. Crane, Warren White, and Peter G. Kramer of Wilmington, NC. The patient reported that he had noticed the facial eruption a couple of months earlier. On examination, the lesion appeared more plaque-like and dense than is typical of rosacea. The differential diagnosis included lupus, mycosis fungoides, rosacea, and angiosarcoma.

A 4-mm punch biopsy was performed (A), and serologic tests for antinuclear, anti–SS-A, and anti–SS-B antibodies were negative. The patient was given prednisone; cephalexin; and fluticasone propionate cream, 0.05%.

The biopsy slide showed the dermis with ectatic anastomosing blood vessels that were lined with atypical endothelial cells. Mitotic figures were noted within the endothelial cells. This confirmed the diagnosis of angiosarcoma, and radiation therapy was begun immediately. There was no sign of angiosarcoma 6 months later (B), but recurrence is expected because this disease is almost always fatal.

Angiosarcoma of the skin (also called malignant angioendothelioma) is rare. There are two types: angiosarcoma secondary to chronic lymph edema, or Stewart-Treves syndrome; and angiosarcoma of the face and scalp of the elderly. This patient had angiosarcoma of the face, which was first described by Caro and Stubenrauch in 1945.1 These tumors metastasize to cervical lymph nodes, liver, and lungs and can invade the orbital bones. Treatment options include wide surgical excision and Mohs' surgery for primary lesion, and radiotherapy, which is considered palliative, for both primary lesions and metastases.

REFERENCE:1. Caro MR, Stubenrauch CH Jr. Hemangioendothelioma of the skin. Arch Dermatol Syph. 1945;51:295-304.