Genetic alterations in salivary gland cancers

Abstract

Salivary gland cancers are an incredibly heterogeneous group of tumors that include 24 histologically distinct tumor types. The use of new genetic methods has paved the way for promising advancements in our understanding of the molecular biology underlying each type of tumor. The objective of this review was to highlight common oncogenes, tumor suppressor genes, and cytogenetic and epigenetic changes associated with the most common tumor types: mucoepidermoid carcinoma, adenoid cystic carcinoma, salivary duct carcinoma, mammary analogue secretory carcinoma, hyalinizing clear cell carcinoma, carcinoma ex pleomorphic adenoma, and acinic cell carcinoma. Recent insights into the pathogenesis of each cancer subtype have helped better define and classify these tumors. Further research in salivary gland cancers should focus on determining the key genes involved in the tumorigenesis of each distinct malignancy and identifying individualized chemotherapies directed at these targets.

Significant and durable clinical benefit from trastuzumab in 2 patients with HER2‐amplified salivary gland cancer and a review of the literature

“…Salivary gland cancers are a heterogeneous group of rare head and neck tumors with several histologically distinct subtypes, including mucoepidermoid carcinoma, adenoid cystic carcinoma (ACC), salivary duct carcinoma, mammary analog secretory carcinoma, and CEPA . Of these subtypes, mucoepidermoid carcinoma is the most common, accounting for 30% to 35% of cases, whereas CEPA and salivary duct carcinoma comprise 10% to 15% and 5% to 10% of cases, respectively .…”

Salivary Gland Neoplasms: Does Morphological Diversity Reflect Tumor Heterogeneity

“…The t(11; 19)(q21;p13) translocation involving CRTC1-MAML2 genes has been described in 38-81% of MEC cases and, although its presence is usually associated with a better prognosis [40], coexistence of the CDKN2A deletion is associated with advanced-stage lethal disease [41]. A minority may also express, in a mutually exclusive way, the translocation t(11; 15)(q21;q26) leading to a CRTC3-MAML2 fusion [42].…”

Secretory carcinoma — impact of translocation and gene fusions on salivary gland tumor

“…In the salivary gland, four cancers have been reported harboring recurrent translocations (28,29). These include adenoid cystic carcinoma (AdCC), hyalinizing clear cell carcinoma (HCCC), MEC, and the newly identified SC (11,28) ( Table 2).…”

“…A disease-defining translocation in the Ewing sarcoma RNA-binding protein 1 (EWSR1) gene was identified in more than 80% of HCCCs (33). A t(12;22)(q13;q12) translocation produces the most common fusion transcript consisting of the genes EWSR1 and activating transcription factor 1 (ATF1) (28).…”