Adenoid cystic carcinoma of the breast

Adenoid cystic carcinoma (ACC) is a rare histological form of the breast cancers. Due to the rarity of this type of carcinoma, the therapeutic management strategy still controversial. A retrospective study reporting data concerning five patients with breast ACC diagnosed between 1993 and 2017. Cases were reviewed for age at diagnosis, clinical, radiological and histological tumour presentation, type of treatment and treatment outcomes. Out of a total of 3066 breast cancer patients, 5 patients had breast ACC (0.1%). The median age at diagnosis was 46 years (range 26-57 years). A palpable mass was the common presenting symptom in all cases. No patient had clinical evidence of regional lymph node metastases and staging investigations showed no evidence of metastatic disease. All patients underwent primary surgery with ipsilateral axillary lymph node dissection in 3 cases. Adjuvant chemotherapy was performed in two patients and postoperative radiotherapy in all cases. Tamoxifen-based hormonal therapy was administered in an only patient who had positive hormonal receptors. After a median follow-up of 107 months, the overall mean survival was 106.8 months and the average disease-free survival was 97.8 months. Breast ACC has a low propensity for local recurrence after excision despite triple negative nature. Since the frequency of axillary lymph node metastases is very low, we suggest that lymphadenectomy is to be avoided in this type of tumour. Adjuvant radiotherapy remains a standard treatment after conservative surgery.