Lymphangiosarcoma following mastectomy.

This study reviews 16 cases of lymphangiosarcoma of the upper extremity after mastectomy for breast cancer (Stewart-Treves syndrome) was done at the author's institution from 1970 to 1992. Lymphangiosarcoma was diagnosed an average of 10.6 years (range, 5.6-18 years) after the diagnosis of breast cancer. Presenting signs included a bruise (6 patients); increased swelling (4); a red, raised lesion (2); a palpable mass (2); a blister appearance (1); and a non-healing eschar with continual bleeding (1). After biopsy, surgical treatment included forequarter amputation (8 patients), wide excision with grafting (5), and above-elbow amputation (1). Local recurrence of the lymphangiosarcoma occurred on the chest wall in 11 of the 16 patients after an average of 10.9 months. Metastasis occurred in 11 patients. (Metastasis occurred in 1 patient without local recurrence, and 1 patient with locally recurrent lymphangiosarcoma died of breast cancer before evidence of metastatic lymphangiosarcoma.) There are 2 long-term survivors who have lived 9.3 and 6 years, respectively, since the operation. One had been treated with a forequarter amputation and the other with wide excision. Early recognition and surgical treatment seem to offer the only chance for long-term survival.