Abstract

Adenoid cystic carcinoma (ACC) of the breast is a rare subtype of breast cancer. Despite its triple-negative status, this tumor has a very good prognosis. Breast-conserving treatment including postoperative radiotherapy seems to be equivalent to mastectomy alone with respect to survival. Routine axillary lymph node staging is not recommended since lymph node metastases are extremely rare. The value of adjuvant systemic therapies is not established. Late relapses can occur, so long-term follow-up is mandatory for these patients.

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