Clinicopathological features and prognostic analysis of 30 patients with laryngeal and hypopharyngeal adenoid cystic carcinoma: a single-center retrospective study.

PURPOSE: To evaluate clinical characteristics, treatment outcomes, and prognostic factors in patients with laryngeal and hypopharyngeal adenoid cystic carcinoma (LHACC).

METHODS

This retrospective, single-institution study (2013–2023) included patients with pathologically confirmed LHACC who underwent primary surgical treatment. Clinical and pathological variables were reviewed, and relevant gene alterations were extracted from cBioPortal. Overall survival (OS) and disease-free survival (DFS) were estimated using Kaplan–Meier curves and compared by the log-rank test.

RESULTS

Thirty patients were analyzed (median follow-up, 36 months; range, 4–130). The cohort comprised 10 men and 20 women (mean age 51 years). Hoarseness (36.7%) was the most frequent symptom and correlated with poorer OS (P = 0.033). Locoregional recurrence occurred in 5 patients (16.7%), and distant metastases in 15 (50%). OS rates at 3, 5, and 10 years were 95.5%, 76.4%, and 38.2%; DFS rates were 79.2%, 49.4%, and 10.3%. Survival rates were estimated using the Kaplan–Meier method, with 9 censored patients who had follow-up shorter than 3 or 5 years without tumor progression or death. Higher Ki-67 index (> 15%) predicted an unfavorable DFS (P = 0.041). Gene fusions (4 MYB–NFIB, 1 CTNNA3–NFIB) were detected in 50% of tested cases, highlighting molecular features of LHACC.

CONCLUSIONS

Hoarseness and Ki-67 may help identify high-risk LHACC patients. Surgical resection remains the cornerstone of therapy, but individualized management and long-term follow-up are essential. Frequent MYB–NFIB fusions underscore the molecular basis of LHACC and may inform future prognostic assessment and therapeutic strategies.