Images in Clinical Medicine
The Stewart–Treves Syndrome
N Engl J Med 2008; 359:950August 28, 2008
- Article
A 67-year-old woman with a history of cancer of the right breast, treated with a modified radical mastectomy, radiation, and chemotherapy in 1990, presented with a 6-month history of an enlarging plaque of coalescing purple papules and nodules within a region of chronic lymphedema of the right upper arm. A skin biopsy of the lesion showed collections of spindled cells with large, vesicular nuclei adjacent to slit like spaces lined by cells with pleomorphic nuclei — findings diagnostic of angiosarcoma. Immunostaining for human herpesvirus 8 was negative. Angiosarcoma arising within chronic postmastectomy lymphedema was reported in 1948 by Stewart and Treves. The lymphedematous limb is an immunologically vulnerable area, susceptible to the development of neoplasms and infections. This condition has a very poor prognosis, with an overall survival rate of 10 to 30%. This patient died after unsuccessful treatment with doxorubicin, amputation of the right arm, and radiation therapy.
Laura B. Pincus, M.D.
Lindy P. Fox, M.D.
University of California at San Francisco, San Francisco, CA 94143
foxli@derm.ucsf. edu
