Primary sarcomas of the breast are extremely rare and heterogenous malignancies; they should be differentiated from phyllodes tumors. They are characterized by their high rate of recurrence, rapid growth and aggressive coarse. We present a case of a 41-year-old Palestinian female who presented with a recurrent breast mass. Biopsy showed primary sarcoma and imaging confirmed metastasis to axillary lymph nodes. The patient received six cycles of ifosfamide-adriamycin protocol resulting in complete pathological response. She underwent left sided modified radical mastectomy followed by radiation and six more cycles of the same protocol as adjuvant. Primary breast sarcomas are rare neoplasms that require multidisciplinary discussion to guide treatment. The approach to these tumors is chemotherapy followed by surgical resection when operable, in addition to local control via radiotherapy and adjuvant chemotherapy.
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