Treatment of Recurrent or Metastatic Adenoid Cystic Carcinoma of the Submandibular Gland

Adenoid cystic carcinoma (AdCC) is the most common malignant tumour type of the submandibular gland. When disease recurs or spreads, it presents a distinct clinical challenge requiring systemic management.

Clinical Scenario

This protocol addresses patients with adenoid cystic carcinoma of the submandibular gland with recurrent or metastatic disease. Metastatic AdCC is generally characterised by multiple locoregional recurrences accompanied by distant metastases in about half of cases.

Treatment Approach

Systemic treatment options in this setting are guided by tumour biology and disease extent. The approach may include angiogenesis-targeting agents or cytotoxic regimens, and certain molecular findings can direct patients toward specific clinical trial pathways.

The complete evidence-based regimen — including specific agent selection, sequencing, and additional clinical considerations — is available in the full structured protocol.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1016/j.esmoop.2022.100602

The most common submandibular gland malignant tumour type is AdCC.

Metastatic AdCC is generally characterised by multiple locoregional recurrences accompanied by distant metastases in about half of cases.

When starting systemic treatment, consider angiogenesis inhibitors [III, C] or platinum-based ChT (i.e. CAP or doxorubicin-cisplatin) [III, B].

In case of activated NOTCH mutation, participation in a NOTCH inhibitor clinical study is recommended, if possible [V, B].

In case of liver and/or bone involvement, there is a lower threshold to start (often more aggressive) systemic treatment [IV, C].

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