Adenoid Cystic Carcinoma of the Breast A focused review

Adenoid cystic carcinoma is the most common epithelial malignancy of the minor salivary glands, where it follows an aggressive course with a strong predilection for metastases; by contrast, breast adenoid cystic carcinoma (bACC) represents only 0.1% of breast malignancies and has an indolent natural history with metastases occurring as an uncommon event. Identification of bACC is challenging, as patient risk factors, clinical presentation, and imaging features on ultrasound and MRI are similar to other breast malignancies. Histopathological evaluation is, therefore, critical to the accurate diagnosis of bACC. Additionally, despite being a triple negative hormonal breast cancer, bACC is relatively indolent with excellent overall survivorship; however, delayed metastases are reported up to ten years following initial diagnosis and therefore longterm surveillance is strongly recommended. This manuscript provides an overall Focused Review on the epidemiology, clinical features, diagnostic workup, pathogenesis, treatment and prognostic/predictive factors of bACC as currently available in the published literature.