Version July 2025
ACC: adenoid cystic carcinoma; CAP: cyclophosphamide, doxorubicin, cisplatin; dMMR: mismatch repair deficient; HER2: human epidermal growth factor receptor 2; MASC: mammary analog secretory carcinoma; MEC: mucoepidermoid carcinoma; MSI-H: microsatellite instability high; NOS: not otherwise specified; NTRK: neurotrophic tyrosine receptor kinase; RET: rearranged during transfection; SDC: salivary ductal carcinoma; TMB: tumor mutational burden.
* 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.
Options include:◊ 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.
¥ Upon progression of initial chemotherapy, chemotherapy with other unused treatment options may be offered.