Stewart-Treves syndrome. A lethal complication of postmastectomy lymphedema and regional immune deficiency.

Lymphangiosarcoma is a fatal complication of postmastectomy lymphedema. The pathogenesis of lymphangiosarcoma in chronic lymphedema is a combination of two factors. First, the edematous region responds in a manner similar to "immunologically privileged sites." Second, because of its anatomic and physiologic properties, it is a favorable site for the development of mutant cell populations for reasons that are not fully understood. As a result, these mutant cells, with their genetically nonidentical antigens, escape recognition by the host's impaired immune surveillance mechanism. The failure to promote a sufficient immune response allows unrestricted tumor growth to take place, resulting in the ultimate death of the patient. Available therapeutic measures are equally unsatisfactory. Emphasis is placed on periodic examination of the lymphedematous extremity, aggressive treatment of established lymphedema and infections, and surgical preservation of lymphatic channels during breast cancer surgery.

Pregnancy and breast cancer.

Breast cancer in association with pregnancy and lactation is rare, but presents a therapeutic problem of considerable magnitude. The outlook for such patients is less favorable than that of nonpregnant, nonlactating women, probably because the stage of the disease is more advanced when it is discovered. The most significant factor in the poorer prognosis is physician delay in diagnosis and therapy. When mastectomy is carried out early in pregnancy, the operation can be as effective as in nonpregnant women of the same age groups. It is emphasized that when pregnancy and breast cancer are found concurrently, prompt therapy for the cancer should be undertaken. Interruption of pregnancy in nondisseminated breast cancer is of little value. If pregnancy is near term when the diagnosis of disseminated breast cancer is made, the desire of the husband and wife for a child should be considered. A modest delay in therapy to allow for delivery probably has no deleterious effect. Castration should be withheld and used only for the patient with metastatic disease. There may be a place for prophylactic castration in the treatment of disseminated disease, but its role is yet to be clearly defined. Subsequent pregnancies in a patient with axillary spread at the time of mastectomy are contraindicated, because of the high rate of treatment failure and decreased rate of survival. In patients desiring future pregnancies following mastectomy, a period of observation of at least 2 years seems wise. At the end of that period, if clinical evaluation, laboratory values, roentgenographic studies, and isotopic bone scanning are negative for disseminated disease, subsequent pregnancies seem safe. Prompt evaluation of any breast mass found during pregnancy and lactation should be carried out by needle or operative biopsies under local anesthesia. Although the prognosis of the pregnant or lactating woman with breast cancer is generally favorable, numerous long-term survivals are encountered in those women who undergo prompt mastectomy early in pregnancy. The former pessimistic outlook for such patients seems unjustified. With modern methods of diagnosis and treatment, therapy can be effective and successful.