Systemic therapy of metastatic or recurrent salivary gland tumors

ACC: adenoid cystic carcinoma; MASC: mammary analog secretory carcinoma; NTRK: neurotrophic tyrosine receptor kinase; MEC: mucoepidermoid carcinoma; SDC: salivary ductal carcinoma; RET: rearranged during transfection; NOS: not otherwise specified; HER2: human epidermal growth factor receptor 2; dMMR: mismatch repair deficient; MSI-H: microsatellite instability high; TMB: tumor mutational burden; CAP: cyclophosphamide, doxorubicin, cisplatin.

* There is no standard approach to surveillance. Refer to UpToDate content on treatment of metastatic salivary gland tumors for suggested surveillance protocols.

¶ Locoregional therapy (eg, surgical resection, radiofrequency ablation, radiation therapy) may be considered in patients with a solitary site of metastasis or one symptomatic site of disease affecting a critical structure with otherwise stable metastatic disease.

Δ For those eligible for systemic chemotherapy, combination chemotherapy is preferred over single-agent therapy to maximize treatment response.

• CAP
• Mitoxantrone
• Vinorelbine
• Cisplatin
• Doxorubicin or epirubicin
• Methotrexate
• Paclitaxel (non-ACC histology)

◊ If MASC histology and NTRK negative, the diagnosis of secretory tumor is in question and second review of histology is necessary, given virtually 100% of MASC tumors have NTRK gene fusions. NTRK mutation-negative tumors should be treated as nonsecretory tumors.

§ Trastuzumab plus pertuzumab is an alternative for those who prefer to avoid the potential toxicities of chemotherapy or have a contraindication to taxanes.