Lymphangiosarcoma arising in chronic lymphedematous extremities

Abstract

Data from 23 cases of lymphangiosarcoma associated with chronic lymphedema seen at the Mayo Clinic and 163 cases from the world literature are reviewed. The available information in 162 cases of postmastectomy lymphedematous lymphangiosarcoma revealed that the lesion occurred at an average age of 63.9 years (range 44 to 84 years) and an average of 10 years 3 months after mastectomy. Fifty per cent of the 129 patients eligible for follow-up were dead within 19 months after treatment, and only 11 patients have survived 5 years or more. Amputation gave a slightly better prognosis than did radiation therapy. Of 24 patients (two from the Mayo Clinic) with lymphangiosarcoma and non-postmastectomy lymphedema, 12 (50%) were dead within 34 months after treatment. Only two of the patients were long-term survivors (5 and 15 years), and both were treated by amputation. Early recognition of the lesion and prompt radical ablative surgery seem to offer the best chance for survival.