Overview

NCI Definition: A malignant tumor arising from the epithelial cells. Microscopically, the neoplastic epithelial cells form cylindrical spatial configurations (cribriform or classic type of adenoid cystic carcinoma), cordlike structures (tubular type of adenoid cystic carcinoma), or solid structures (basaloid variant of adenoid cystic carcinoma). Adenoid cystic carcinomas mostly occur in the salivary glands. Other primary sites of involvement include the lacrimal gland, the larynx, and the lungs. Adenoid cystic carcinomas spread along nerve sheaths, resulting in severe pain, and they tend to recur. Lymph node metastases are unusual; hematogenous tumor spread is characteristic. [1]

Adenoid cystic carcinomas most frequently harbor alterations in NOTCH1, KDM6A, BCOR, TP53, and ARID1A [2].

Most Commonly Altered Genes in Adenoid Cystic Carcinoma

NOTCH1 Mutation, NOTCH1 Frameshift, BCOR Mutation, TP53 Mutation, and KDM6A Mutation are the most common alterations in adenoid cystic carcinoma [2].

Top Alterations in Adenoid Cystic Carcinoma

Significant Genes in Adenoid Cystic Carcinoma

ERBB2 +

NOTCH1 +

NOTCH2 +

NOTCH3 +

NOTCH4 +

TP53 +

Disease Details

Synonyms
Adenocystic Carcinoma, Cylindroid Adenocarcinoma, adenoid cystic cancer
Parent(s)
Carcinoma
Children
Esophageal Adenoid Cystic Carcinoma, Adenoid Cystic Breast Carcinoma, Tracheal Adenoid Cystic Carcinoma, Cervical Adenoid Cystic Carcinoma, Bartholin Gland Adenoid Cystic Carcinoma, Ethmoid Sinus Adenoid Cystic Carcinoma, Lung Adenoid Cystic Carcinoma, Maxillary Sinus Adenoid Cystic Carcinoma, Adenoid Cystic Skin Carcinoma, Laryngeal Adenoid Cystic Carcinoma, Salivary Gland Adenoid Cystic Carcinoma, Prostate Adenoid Cystic Carcinoma, and Lacrimal Gland Adenoid Cystic Carcinoma

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 6. 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.