Lymphangiosarcoma in the lymphedematous arm after radical mastectomy

INCE the first report of six cases of lym-S phangiosarcoma developing in the post-radical-mastectomy lymphedematous arm by Stewart and Treves in 1948, eleven more cases have been reported (Table I), all showing the typical characteristics of this apparently rare phenomenon.With the exception of one case reported by Southwick and Slaughter,l8 all of these cases have developed metastases.It had been thought that this very malignant lesion developed only in the edematous arms of patients after radical mastectomy until the description of a case by Martorell in which the tumor occurred in a lower extremity.Martorell's patient had developed lymphedema after a compound fracture and subsequently developed lymphangiosarcoma.Our cases, however, both followed radical mastectomy in patients with postmastectomy lymphedema.All previously reported instances of this lesion and the two to be described in this paper have presented a characteristic pattern.All followed postmastectomy edema, and the earliest appearance of lymphangiosarcoma followed the original surgery by five years.Most lymphangiosarcomas appeared some nine years after mastectomy.T h e locations varied, some tumors appearing on the forearm and others on the upper arm.In general, the skin was involved first and in only three cases have there been descriptions of extension of the disease beneath the deep fascia of the extremity.By and large, this tumor has been superficial in its original exteni, but distant metastases, mainly to the lungs, have caused death in all cases so far reported except Southwick and Slaughter's.18Treatment has varied from local excision to interscapulothoracic amputation.Roentgen-ray therapy was given in three cases, two patients responding well to this form of treatment and the one