There is no standard-of-care systemic therapy for metastatic adenoid cystic carcinoma (ACC), and its management remains challenging. This study aimed to evaluate treatment patterns and disease outcomes in patients with metastatic head and neck ACC.

We conducted a retrospective analysis of patients diagnosed with metastatic head and neck ACC between January 2005 and December 2023.

A total of 51 patients were included in the study, of whom 36 (70.6%) received at least one line of systemic treatment. The median overall survival (OS) for the entire cohort was 29.21 months. Patients with lung metastases had significantly longer OS compared to those without (44.52 vs. 11.04 months, p = 0.008). The median OS for patients who received at least one line of systemic treatment was 26.78 months. The median progression-free survival (PFS) was 7.82 months, 6.11 months, and 4.53 months for first-, second-, and third-line treatments, respectively. Disease progression was the primary reason for treatment discontinuation across all therapy lines.

There is no consensus on the optimal treatment approach for metastatic ACC, and therapeutic strategies remain heterogeneous. Patients with lung metastases had better survival outcomes than those with metastases at other sites.