Treatment of Recurrent or Metastatic Adenoid Cystic Carcinoma

Purpose of Review Adenoid cystic carcinoma (ACC) is a rare and heterogeneous malignancy of secretory glands. Recurrence after curative-intent treatment is common, and approximately 40% of patients develop metastatic disease, for which consensus is lacking regarding therapeutic approaches. Here, we r...

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Bibliographic Details
Published in:Current oncology reports 2022-05, Vol.24 (5), p.621-631
Main Authors: de Sousa, Luana Guimaraes, Neto, Felippe Lazar, Lin, Jessica, Ferrarotto, Renata
Format: Article
Language:English
Subjects:
Adenoid
Carcinoma, Adenoid Cystic - drug therapy
Carcinoma, Adenoid Cystic - therapy
Clinical trials
Cytotoxic agents
Cytotoxicity
Head and Neck Cancers (EY Hanna
Humans
Malignancy
Medicine
Medicine & Public Health
Metastases
Metastasis
Neoplasm Recurrence, Local - drug therapy
Neoplasm Recurrence, Local - pathology
Oncology
Patients
Prognosis
Section Editor
Therapeutic targets
Topical Collection on Head and Neck Cancers
Vascular endothelial growth factor receptors
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Summary:Purpose of Review Adenoid cystic carcinoma (ACC) is a rare and heterogeneous malignancy of secretory glands. Recurrence after curative-intent treatment is common, and approximately 40% of patients develop metastatic disease, for which consensus is lacking regarding therapeutic approaches. Here, we review the available therapies for recurrent/metastatic (R/M) ACC and offer our perspectives on future treatment options. Recent Findings Proteogenomic studies of ACC revealed two molecular subtypes with therapeutic implications: ACC-I (37% of cases) and ACC-II (63%); each has distinct disease biology and prognosis. Molecular drivers, such as NOTCH1 , have emerged as potential therapeutic targets for ACC-I and are being explored in clinical trials. Summary Despite its biological heterogeneity, treatment for R/M ACC is not personalized and limited to cytotoxic agents and VEGFR inhibitors, which produce modest responses and significant toxicity. The increasing understanding of ACC’s molecular biology might guide the development of biomarkers for patient selection and new therapies development.
ISSN:1523-3790
1534-6269
DOI:10.1007/s11912-022-01233-z