Adenoid Cystic Carcinoma of the Breast: A Narrative Review and Update on Management

Rare breast malignancies represent a challenge for treatment decision making given the lack of evidence-based guidelines. As a particularly uncommon tumor, adenoid cystic carcinomas are especially challenging. Although, histopathologically, they share the same tumor molecular profile as hormone receptor-negative and HER2 nonamplified carcinomas with aggressive physiology, adenoid cystic carcinomas generally have a favorable prognosis. Thus, there is evidence to suggest that more aggressive treatment regimens may not provide better therapeutic effects. In this review, we discuss ACCB with the goal of highlighting pathophysiology, clinical features, and treatment strategies. Existing data support consideration for adjuvant radiation with the omission of axillary staging and systemic therapies.