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February 1967

Lymphangiosarcoma: A Lethal Complication of Chronic Lymphedema: Report of Two Cases and Review of the Literature

Arch Surg. 1967;94(2):223-230. doi:10.1001/archsurg.1967.01330080061017

IN 1948, Stewart and Treves1 described six cases of a previously unrecognized clinicopathological entity—lymphangiosarcoma in postmastectomy lymphedema. Eighty-eight similar cases2-58 from the literature and three unpublished cases (written communications from W. R. Nelson and W. D. McCarthy, on Jan 26, 1966, and April 29, 1966, respectively) have been variously designated angiosarcoma, angioplastic sarcoma, malignant lymphostatic endothelioma, and lymphangioendothelioma and hemangioendothelioma, reflecting the differences in concept regarding the tumor's histogenesis. In addition, 15 cases10,19,30,33,50,59-69 of lymphangiosarcoma arising in primary or secondary lymphedematous extremities unassociated with breast carcinoma have been reported.

This is a report of two postmastectomy lymphangiosarcomas (Stewart-Treves syndrome) studied at the Henry Ford Hospital since 1960 and a review of the literature, including follow-up information on 19 of the 112 cases.

Report of Cases 

Case 1.  —A 68-year-old white woman had lymphedema of the right arm for 18 years following a radical mastectomy and preoperative and