D.
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C.D.
Diagnosis: Angiosarcoma
Histopatholgy: Revealed a dermal infiltrate of atypical cells forming anastomosing channels lined by plump protuberant cells with hyperchromatic nuclei (Figs. D,E) Specials stains were positive for CD 31 and vimentin (not shown).
Discussion: Cutaneous angiosarcoma is a rare vascular tumor that most commonly involves the upper part of the face and scalp of elderly people. Men are affected more frequently than women. Lesions have been described to rarely arise in the setting of chronic lymphedematous extremities. In 1948, Stewart and Treves described a syndrome of postmastectomy lymphangiosarcomas occurring in the edematous upper extremities of women who had undergone radical mastectomies. Angiosarcomas have also rarely been described as occurring in areas of previous radiotherapy for both benign and malignant conditions.
Angiosarcomas can present as single or multifocal, erythematous to violaceous nodules, plaques, or flat infiltrating areas. They occasionally bleed or ulcerate.
Histologically, lesions appear as dermal tumors that infiltrate subcutaneous fat and may have a multifocal distribution. There is a meshwork of anastomosing dilated vessels which extends between the dermal collagen bundles and around skin appendages. The vessels are irregular and usually lined by atypical endothelial cells that are plump protuberant cells with enlarged hyperchromatic nuclei. CD 31 is a relatively sensitive and specific immunohistochemical marker for endothelial differentiation. Unfortunately, the more poorly differentiated tumors are less likely to stain with markers. Because angiosarcomas have aggressive local growth patterns, the margins are difficult to define surgically. Even after multiple surgical excisions of the primary tumor, metastasis to regional lymph nodes and the lungs is common. The prognosis is poor. One series found that only 15% of the patients survived 5 years or more after the diagnosis was made.
Course: Our patient is currently being evaluated by a tumor board to determine her best therapeutic options.
References:
1. Holden CA, Spittle MF, Wilson JE. Angiosarcoma of the face and scalp, prognosis and treatment. Cancer 59: 1046-1057; 1987.
2. Weedon D. Skin Pathology. Churchill Livingstone, pp. 845-847; 1997.
3. Fitzpatrick's Dermatology in General Medicine. Freedberg IM, Eisen AZ, Wolff K, et al (eds). 5th Ed. New York, McGraw-Hill, p. 1715; 1999.
My thanks to Drs. Timothy Storer and Lester Reese for their help in the preparation of this case.
