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19-07-2022 | Systemic Therapy | Head and Neck Cancer (PL Swiecicki, Section Editor)

Metastatic Adenoid Cystic Carcinoma: Genomic Landscape and Emerging Treatments

Authors: Luana Guimaraes de Sousa, Katarina Jovanovic, Renata Ferrarotto

Published in: Current Treatment Options in Oncology | Issue 8/2022

Opinion statement

Adenoid cystic carcinoma (ACC) is a heterogeneous cancer that commonly develops in the salivary glands. Approximately 40 to 50% of patients with ACC develop recurrence and/or metastasis. Although most patients with ACC have slow-growing disease, a subset experiences aggressive disease with early visceral and/or bone metastasis. Thus far, there is no consensus on the best time to start palliative treatment in patients with indolent disease. The only systemic therapies available for recurrent or metastatic ACC are cytotoxic agents and multikinase inhibitors targeting vascular endothelial growth factor receptor, and both types of therapy have modest activity. Studies integrating proteomics, genomics, and clinical data have revealed distinct molecular ACC subtypes, ACC-I and ACC-II, with ACC-I generally associated with more aggressive disease biology. ACC-I tumors were enriched for NOTCH1-activating mutation and upregulation of MYC and MYC targets, while ACC-II tumors exhibited upregulation of TP63 and receptor tyrosine kinases. These findings highlight the importance of patient selection for surveillance and targeted therapy development in ACC. In recent clinical trials of targeted therapy in ACC, patients are being selected according to tumor molecular profile (e.g., presence of NOTCH-activating mutations), which represents a major advance in the field. Ongoing collaborative research focusing on the development of novel therapeutic strategies for ACC patients based on disease biology will increase the drug armamentarium and improve survival outcomes for these patients in dire need.

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Title: Metastatic Adenoid Cystic Carcinoma: Genomic Landscape and Emerging Treatments
Authors: Luana Guimaraes de Sousa
Katarina Jovanovic
Renata Ferrarotto
Publication date: 19-07-2022
Publisher: Springer US
Keywords: Systemic Therapy
Targeted Therapy
Axitinib
Lenvatinib
Salivary Gland Cancer
Salivary Gland Cancer
Published in: Current Treatment Options in Oncology / Issue 8/2022
Print ISSN: 1527-2729
Electronic ISSN: 1534-6277
DOI: https://doi.org/10.1007/s11864-022-01001-y

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