Salivary glands adenoid cystic carcinoma: a molecular profile update and potential implications

Adenoid cystic carcinoma (ACC) is an aggressive tumor with a high propensity for distant metastasis and perineural invasion. This tumor is more commonly found in regions of the head and neck, mainly the salivary glands. In general, the primary treatment modality for ACC is surgical resection and, in some cases, postoperative radiotherapy. However, no effective systemic treatment is available for patients with advanced disease. Furthermore, this tumor type is characterized by recurrent molecular alterations, especially rearrangements involving the MYB, MYBL1, and NFIB genes. In addition, they also reported copy number alterations (CNAs) that impact genes. One of them is C-KIT, mutations that affect signaling pathways such as NOTCH, PI3KCA, and PTEN, as well as alterations in chromatin remodeling genes. The identification of new molecular targets enables the development of specific therapies. Despite ongoing investigations into immunotherapy, tyrosine kinase inhibitors, and anti-angiogenics, no systemic therapy is approved by the FDA for ACC. In this review, we report the genetic and cytogenetic findings on head and neck ACC, highlighting possible targets for therapeutic interventions.

Automated summary

Adenoid cystic carcinoma (ACC) is an aggressive tumor commonly found in the head and neck region. Surgical resection is the primary treatment, but there is no effective systemic therapy for advanced disease. Molecular alterations involving genes such as MYB, MYBL1, and NFIB, as well as copy number alterations (CNAs), have been identified in ACC. Despite ongoing research, there are currently no FDA-approved systemic therapies for ACC.