Adenoid Cystic Carcinoma from the salivary and lacrimal glands and the breast: Different clinical outcomes to the same tumor

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.

Keywords: Adenoid Cystic Carcinoma; Breast; Cancer prognosis; Lacrimal gland; Salivary gland.

Publication types

  • Review

MeSH terms

  • Carcinoma, Adenoid Cystic* / diagnosis
  • Carcinoma, Adenoid Cystic* / genetics
  • Carcinoma, Adenoid Cystic* / therapy
  • Eye Neoplasms* / diagnosis
  • Eye Neoplasms* / pathology
  • Eye Neoplasms* / therapy
  • Humans
  • Lacrimal Apparatus Diseases* / diagnosis
  • Lacrimal Apparatus Diseases* / genetics
  • Lacrimal Apparatus Diseases* / therapy
  • Lacrimal Apparatus* / pathology
  • Salivary Gland Neoplasms* / diagnosis
  • Salivary Gland Neoplasms* / genetics
  • Salivary Gland Neoplasms* / therapy