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 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. 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.

Automated summary

In this review, the available therapies for recurrent/metastatic (R/M) adenoid cystic carcinoma (ACC) were discussed. Recent studies have revealed two molecular subtypes of ACC, which offer potential therapeutic targets. However, the current treatment for R/M ACC is not personalized and limited, and there is a need for biomarkers and new therapies development.