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CA: A Cancer Journal for Clinicians, Vol 31, 284-299, Copyright © 1981 by American Cancer Society

Lymphangiosarcoma in Postmastectomy Lymphedema: A Report of Six Cases in Elephantiasis Chirurgica

¹ Attending Pathologist, New York Hospital (1949-1959); Attending Pathologist and Head of the Department of Pathology, Memorial Hospital for Cancer and Allied Diseases; and Professor of Pathology, Cornell University Medical School (1936-1959).
² Attending Surgeon on the Breast Service of Memorial Hospital for Cancer and Allied Diseases (1952-1960) and as Associate Professor of Clinical Surgery at Cornell University Medical School (1953-1960).

The basis for the development of tumors of the type described in lymphedematous arms following radical mastectomy is naturally obscure. One would like to speculate upon the existence of a systemic carcinogenic factor responsible not only for the lymphangioblastic tumors but likewise for the initial mammary cancers, and in the one case for the squamous carcinoma of the skin. It would surely be remarkable were the lymphedema alone the predisposing factor, for in other diseases involving chronic lymphedema, e.g., filariasis, one does not find these tumors reported. In our experience they are peculiar to the obstruction following radical mastectomy. They have occurred without axillary metastases of the breast cancer, with or without postoperative irradiation, and arise in sites where no irradiation has been administered. Hence the hypothesis of a systematic carcinogen is attractive, something acting in a locus minoris resistentiae. Unfortunately, however, in the case of one patient we have no confirmation pathologically of the existence of the breast cancer. If we accept the clinical diagnosis in this case, the correlation (breast cancer, lymphedema, lymphangioblastic sarcoma and in one instance likewise squamous cancer, together with the absence of similar tumors in unrelated types of lymphedema) renders it impossible to discard the thesis proposed—that of the existence of a systematic carcinogen.

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