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Treatment of Recurrent or Metastatic Adenoid Cystic Carcinoma

  • Head and Neck Cancers (EY Hanna, Section Editor)
  • Published:
Current Oncology Reports Aims and scope Submit manuscript

Abstract

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.

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We thank Stephanie Deming, scientific editor, Research Medical Library, for editing this article.

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  1. Departments of a Thoracic/Head & Neck Medical Oncology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd, PO Box 432, Houston, TX, 77030, USA

    Luana Guimaraes de Sousa, Felippe Lazar Neto, Jessica Lin & Renata Ferrarotto

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Luana Guimaraes de Sousa declares that she has no conflict of interest. Felippe Lazar Neto declares that he has no conflict of interest. Jessica Lin declares that she has no conflict of interest. Renata Ferrarotto is supported, in part, by grants from the Adenoid Cystic Carcinoma Research Foundation, KLUS Pharma, and Ayala Pharmaceuticals; has received compensation for service as a consultant from Bicara Therapeutics, Carevive Systems, Intellisphere, and Guidepoint; has received speaker’s honoraria from Medscape; has receive payment for expert testimony from Merck; has participated on Data Safety Monitoring or advisory boards for Regeneron/Sanofi, Ayala Pharmaceuticals, KLUS Pharma, G1 Therapeutics, and Prelude Therapeutics; and has received non-financial support (equipment, materials, drugs, etc.) from Ayala Pharmaceuticals

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de Sousa, L.G., Neto, F.L., Lin, J. et al. Treatment of Recurrent or Metastatic Adenoid Cystic Carcinoma. Curr Oncol Rep 24, 621–631 (2022). https://doi.org/10.1007/s11912-022-01233-z

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