Treatment of Recurrent or Metastatic Adenoid Cystic Carcinoma.

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. 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. 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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© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.