Adenoid cystic carcinoma of the breast: a case series of 26 patients

Although triple-negative, adenoid cystic carcinoma of the breast (ACCB) is an uncommon, slow-growing malignancy with a favorable prognosis. There are no treatment standards or therapeutic consensus because of its scarcity.

Data on 26 patients with ACCB from two distinct French cancer centers were presented in this study. Clinical, histological and molecular features of these carcinomas were described. Therapeutic management and patient follow-up was also reported.

The median age of this cohort was 59 years [22–82] and the median follow-up was 4.2 years [0.5–23.9]. The most represented stages were IA (45.5%) and IIA (31.8%), with T1-T2 representing nineteen cases (86.3%). Four patients had axillary node metastases. Only 3 tumors were ER or PR positive. All tumors were HER-2 negative. Nine patients had a rearrangement of the MYB gene. Twenty-five patients had initial surgery. Adjuvant chemotherapy was administered to three patient. Twenty patients received adjuvant radiotherapy. At last follow-up, five of the patients had died from complications of the disease.

Although ACCB generally has a good prognosis, the treatment of this rare type of breast cancer remains challenging to ensure favorable outcomes with minimal sequelae. Initial staging and long-term follow-up using appropriate imaging modalities are essential for selecting tailored treatment strategies and detecting early relapses.