Overview

NCI Definition: An adenoid cystic carcinoma arising from the salivary gland. It is characterized by the presence of epithelial and myoepithelial cells forming tubular, cribriform, and solid patterns. It usually presents as a slow growing mass. Patients may experience pain because of the tumor propensity for perineural invasion. The tumor may follow an aggressive clinical course with recurrences and mestastases to distant sites including lungs, bones, brain, and liver. [1]

Salivary gland adenoid cystic carcinomas most frequently harbor alterations in NOTCH1, BCOR, KDM6A, ARID1A, and NFIB [2].

NOTCH1fs, NOTCH1 Mutation, BCOR Mutation, TP53 Mutation, and MYB-NFIB Fusion are the most common alterations in salivary gland adenoid cystic carcinoma [2].

Disease Details

References

1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 8. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.