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Angiosarcoma of the breast is a rare and highly malignant neoplasm with few long-term survivors. Little is known about the effects of chemotherapy for patients with disseminated disease or its role in the adjuvant setting. We report the case of a patient with metastatic angiosarcoma of the breast who achieved a long-term clinical and pathological remission after treatment with methotrexate. The 30-year course of her disease is the longest reported survival with documented metastatic angiosarcoma of the breast. Analysis of the role of chemotherapy in the adjuvant setting and its effects in relation to histological subtype is undertaken. Adjuvant chemotherapy for patients with poorly differentiated angiosarcoma of the breast results in a higher proportion of patients relapse-free (29.2%) compared to patients not receiving adjuvant chemotherapy (4.4%) (p < 0.05). Patients with well-differentiated tumors do not appear to derive benefit from adjuvant chemotherapy. The effects for patients with disseminated disease are reported.

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