University of Cincinnati Medical Center, Department of Radiology, Cincinnati, OH, USA.
University of Cincinnati Medical Center, Department of Pathology, Cincinnati, OH, USA.
J Breast Imaging. 2022 Dec 11;4(6):625-631. doi: 10.1093/jbi/wbac045.
Adenoid cystic carcinoma (ACC) is an uncommon malignancy occurring most frequently in the salivary glands. Breast ACC is rare. Pain is common at the site of ACC; otherwise, presentation is similar to other primary breast cancers. Adenoid cystic carcinomas classically lack calcifications; the imaging manifestations of ACC are otherwise highly variable, likely related to multiple pathologic growth patterns. While ACC in other regions of the body tends to be more aggressive, ACC involving the breast typically has less aggressive biologic characteristics. Classic-type breast ACC has a lower tendency to recur locally with radiation, metastasize to regional lymph nodes, and spread to other parts of the body. Perineural spread of disease can be seen but is not common. The rarer solid basaloid-type has a higher tendency for local or distant spread and recurrence. Although ACC is usually triple receptor-negative (estrogen receptor, progesterone receptor, human epidermal growth factor-2 receptor), the indolent nature of this tumor dictates its management. With classic-type ACC, the inclusion of axillary surgery has no consensus and the use of chemotherapy or hormonal therapy is rare. Axillary nodal surgery and chemotherapy are often included in management of the more aggressive solid basaloid-type. An understanding of the breast imaging, histopathology, and clinical course is key for appropriate treatment and follow-up of ACC.
腺样囊性癌（ACC）是一种罕见的恶性肿瘤，最常发生于唾液腺。乳腺 ACC 较为罕见。ACC 部位常见疼痛；否则，其表现与其他原发性乳腺癌相似。腺样囊性癌通常缺乏钙化；其影像学表现高度可变，可能与多种病理生长模式有关。虽然身体其他部位的 ACC 往往更具侵袭性，但乳腺 ACC 通常具有侵袭性较弱的生物学特征。经典型乳腺 ACC 局部复发、转移至区域淋巴结和扩散至身体其他部位的倾向较低。虽然可以看到神经周围扩散的疾病，但并不常见。更罕见的实性基底样型具有较高的局部或远处扩散和复发倾向。虽然 ACC 通常三受体阴性（雌激素受体、孕激素受体、人表皮生长因子 2 受体），但该肿瘤的惰性决定了其治疗方法。对于经典型 ACC，是否包括腋窝手术尚无共识，且很少使用化疗或激素治疗。更具侵袭性的实性基底样型通常包括腋窝淋巴结手术和化疗。了解乳腺影像学、组织病理学和临床病程对于 ACC 的适当治疗和随访至关重要。
