Rare laryngeal tumors: A retrospective bicentric study on 74 patients and systematic review

Head and neck cancers are the seventh most common malignancy worldwide, with laryngeal cancer (LC) the most prevalent subtype. Although squamous cell carcinoma (SCC) represents 85-90% of LC, rare non-SCC tumors-including laryngeal sarcomas (LS), neuroendocrine carcinomas (LNC), malignant salivary gland tumors (LMSGT), and mucosal melanomas (LMM)-are uncommon but clinically challenging. We combined a bicentric retrospective cohort with a systematic literature review to provide an integrated assessment of these entities.

Patients with non-SCC malignant laryngeal tumors treated with curative intent at two Italian tertiary centers were retrospectively analyzed. Tumors were classified as LS, LNC, LMSGT, or LMM. Primary endpoints were overall survival (OS), disease specific survival (DSS) and recurrence-free survival (RFS). A systematic review contextualized institutional outcomes.

74 patients were included: 34 LS, 25 LNC, 13 LMSGT, and 2 LMM. Surgery was the primary treatment in 98.6 % of cases. LS and LMSGT demonstrated favorable survival, whereas LNC showed poor outcomes (median OS, 2.89 years) and the highest recurrence rate (72 %). Radical tumor excision achieved effective loco-regional control across histologies. LMM prognosis was dismal, with recurrence in both cases. The literature review revealed marked heterogeneity and wide survival ranges, reflecting rarity and biological diversity.

Rare non-SCC laryngeal tumors comprise heterogeneous and often aggressive diseases with variable prognoses. Despite the absence of standardized management, radical surgery provides effective loco-regional control. Integrating bicentric clinical experience with published evidence highlights the need for multicenter collaboration to optimize treatment strategies and improve outcomes in this rare oncologic setting worldwide today.