Management
Recurrent/Metastatic Disease
➤ Patients presenting with metastatic disease may be evaluated for further
treatments such as local ablative treatments or systemic therapy. These
options should be discussed with the patient and will depend on the
patient and tumor factors. (Weak recommendation; IC-L)
➤ In the setting of adenoid cystic carcinoma and/or low grade tumors with
indolent biology with limited metastases (i.e., ≤5 metastases), local
ablative treatments such as surgery (metastatectomy) or stereotactic
body radiation (SBRT) may be offered to delay local disease progression.
(Weak recommendation; IC-L)
➤ Patients may be considered for initiation systemic therapy in the
following circumstances:
» Metastatic deposits are symptomatic and not amenable to palliative local therapy
» Growth has the potential to compromise organ function OR
» Lesions have grown more than 20% in the preceding 6 months.
(Moderate recommendation; IC-L)
➤ For patients with adenoid cystic carcinoma who are candidates for
initiation systemic therapy, a multitargeted tyrosine kinase inhibitor
such as lenvatinib, or sorafenib may be offered if a clinical trial is not
available. (Moderate recommendation; EB-L)
➤ For patients with non-adenoid cystic salivary gland cancer who are
candidates for initiation of systemic therapy, targeted therapy based on
tumor molecular alterations (i.e., AR, HER2, NTRK) may be offered if a
clinical trial is not available. (Moderate recommendation; EB-L)
➤ Cytotoxic chemotherapy combinations may be offered to patients with
symptomatic disease. (Weak recommendation; IC-L)
➤ For patients who are candidates for systemic therapy, checkpoint
inhibitors should not be routinely offered at this time except for patients
with select molecular alteration (high tumor mutational burden, MSI-H).
(Weak recommendation; IC-L)
➤ For patients with histologic tumor types with a high prevalence of
targetable molecular alterations (i.e., AR in salivary duct carcinoma,
NTRK3 in secretory carcinoma) confirmatory target specific testing
should be performed. (Strong recommendation; EB-I)
➤ Patients who may be potential candidates for systemic therapy with
histologic tumor types with low prevalence of targetable molecular
alterations and unknown driver mutation status should be screened
using a comprehensive panel for driver mutations. Patients with driver
mutation negative tumors may then be offered target specific testing
(i.e., AR, NTRK3). (Weak recommendation; EB-L)
