Department of Oral Diagnosis, Piracicaba Dental School, University of Campinas (UNICAMP), Piracicaba, São Paulo, Brazil; Department of Pathology, Faculty of Medical Sciences, University of Campinas (UNICAMP), Campinas, Sao Paulo, Brazil; Department of Periodontics and Oral Medicine, Laboratory of Epithelial Biology, University of Michigan School of Dentistry, Ann Arbor, MI, USA.
Department of Pathology, Faculty of Medical Sciences, University of Campinas (UNICAMP), Campinas, Sao Paulo, Brazil.
Crit Rev Oncol Hematol. 2022 Nov;179:103792. doi: 10.1016/j.critrevonc.2022.103792. Epub 2022 Aug 13.
Adenoid cystic carcinoma (ACC) is a biphasic malignant lesion that can develop at various anatomical sites. Salivary and lacrimal ACC lesions have a high risk of local invasion, metastasis, and poor prognosis. In more distant organs, such as the breast, ACC is a rarer and less aggressive lesion. One of the major predictors of mortality of ACC is perineural invasion, which can be seen in 30 % of breast lesions, 85% of salivary lesions, and almost 100 % of lacrimal gland tumors. The biological differences between these three ACC tumors are still poorly understood. We focused on the current understanding of the genetic variations observed on ACC tumors and prognostic differences associated with distinct anatomical sites. A special effort was made to present the currently available therapies alongside the emerging strategies under development.
腺样囊性癌（ACC）是一种双相恶性病变，可发生在各种解剖部位。唾液腺和泪腺的 ACC 病变具有较高的局部侵袭、转移和预后不良的风险。在更远的器官，如乳房，ACC 是一种罕见的侵袭性较低的病变。ACC 死亡的主要预测因素之一是神经周围侵犯，在 30%的乳房病变、85%的唾液腺病变和几乎 100%的泪腺肿瘤中可见。这三种 ACC 肿瘤之间的生物学差异仍知之甚少。我们重点介绍了目前对 ACC 肿瘤观察到的遗传变异的理解，以及与不同解剖部位相关的预后差异。特别努力介绍了目前可用的治疗方法以及正在开发的新策略。
