Article Details

Adenoid cystic carcinoma (ACC) is a rare, indolent tumor of the breast, accounting for approximately 0.1% to 1% of all breast cancers. The most common presenting complaint is a painful breast mass and the mean age at the time of diagnosis is 50-60 years. Despite its “triple negative” status (lacking estrogen receptor, progesterone receptor, and Her2/neu expressions), ACC of the breast has a favorable prognosis, in contrast to its aggressive nature at other anatomical sites. Radiological appearances of ACC are generally non-specific; the diagnosis can be made on fine needle aspiration cytology. Although surgery upfront is the cornerstone in treatment, the optimal adjuvant therapy remains uncertain due to the tumor’s low incidence. Limited evidence exists regarding the efficacy of chemotherapy, radiation therapy, and hormonal treatments in the adjuvant setting. In the current state of knowledge, results of breast-conserving treatment with postoperative radiation therapy seem to be equivalent to mastectomy alone. Our case series describes five such cases from three institutions, succinctly outlining the clinical characteristics, diagnostic challenges, and treatment considerations related to ACC of the breast. We also review the current literature and emphasize the need for further large-scale research to enhance understanding and inform clinical decision-making for this rare entity.

Keywords: Adenoid cystic carcinoma; Triple negative breast cancer; Chemotherapy; Radiation; Mastectomy