Forty-four cases of
lymphangiosarcoma treated at Memorial Sloan-Kettering
Cancer Center were reviewed. With the exception of four patients with primary
lymphangiosarcoma of the scalp, all patients had had chronic
lymphedema of the involved limb for many years, usually following
mastectomy for
breast carcinoma, but occasionally due to other cases. Although chronic
lymphedema could be implicated in the etiology of
lymphangiosarcoma in all patients with
neoplasms of an extremity, a significant number of patients did not have a history of
radiation therapy at the site where their
tumor developed. Histologically, although there were no differences in the
lymphangiosarcomas between any of the groups of patients, the morphology of the
tumors was influenced in the individual patient by the size and anatomic site of the
sarcoma. Early
amputation seemed to give the best chance of long-term survival, with responses to wide resection,
chemotherapy, and
radiation therapy generally only of short duration.
Amputation after local recurrence was ineffective in preventing pulmonary
metastases and death. At present, early
amputation appears to be the treatment of choice for patients with
lymphangiosarcoma.