The prevalence of primary breast sarcoma is low, occurring in fewer than 1% of women with breast malignancies. The purpose of this study was to examine the presentation, treatment, and prognosis of patients presenting with these neoplasms.

This was a retrospective review of patients with a primary breast sarcoma treated at Mayo Clinic, Rochester, Minnesota, between 1975 and 2001. Follow-up information was obtained.

Of the 55 patients, 17 had breast-conserving therapy and 38 women had mastectomy. The mean patient age at presentation was 52 years (range 22 to 82). The types of sarcoma included angiosarcoma (18), malignant fibrous histiocytoma (11), stromal sarcoma (8), liposarcoma (4), leiomyosarcoma (4), dermatofibrosarcoma protuberans (4), osteosarcoma (3), fibrosarcoma (2), and rhabdomyosarcoma (1). Follow-up information was available for 53 patients, with a mean follow-up of 81 months. Twenty-nine of 53 patients (55%) developed recurrent sarcoma, and 23 patients (43%) died of their disease. Twenty-seven patients had no evidence of recurrence, and 3 patients were alive with disease at last follow-up. Overall median survival of patients with breast sarcoma was 58 months. Patients with angiosarcoma had a poorer outcome than other sarcoma patients. Twelve of 18 patients (67%) died of angiosarcoma, compared with 11 of 32 patients (34%) of all other sarcoma patients combined. Of 34 patients who did not receive adjuvant chemotherapy or radiation, 13 died of their disease (38%), as compared with 10 of 16 patients (63%) who did receive adjuvant therapy.

While primary nonphylloides breast sarcomas are rare tumors, their treatment and prognosis are poor. Adjuvant chemotherapy and radiation did not improve survival in this report. Surgical extirpation remains the only effective treatment.