Lymphoepithelial carcinoma of larynx and hypopharynx: a systematic review and pooled analysis

Purpose: To define the oncologic outcomes and the prognostic factors of laryngeal and hypopharyngeal lymphoepithelial carcinoma (LEC).

Methods: A systematic review and individual patient data analysis were performed according to the PRISMA guidelines. A total of 41 patients (males: 37; mean age: 63.1, standard deviation: 10.0) were included.

Results: The median follow-up time was 36.0 months (interquartile range, IQR: 11.5-73.5), while the median overall survival (OS) time was 96 months (IQR: 39.0-120.0). Estimated disease-specific survival (DSS) rates (95% Confidence Interval, CI; number still at risk) at 1, 3, and 5 years were 94.4% (79.5-98.6; 31), 82.7% (62.7-92.5; 21), and 78.1% (56.8-89.7; 13), respectively. Estimated disease-free survival (DFS) rates (95% CI; number still at risk) at 1, 3, and 5 years were 92.1% (77.4-97.4; 31), 74.9% (55.6-86.8; 21), and 66.6% (46.1-80.8; 13), respectively. Patients undergoing primary surgery had a reduced chance of death from cancer than patients receiving primary radiotherapy (5-year DSS 85.6 vs. 56.2%; p = 0.04), while EBV positivity tend to be a negative prognostic factor (5-year DSS 100 vs. 66.7%; p = 0.05).

Conclusions: Laryngeal and hypopharyngeal LEC is a rare disease with favorable survival. Further prospective multicenter studies are needed to make a reliable statement on prognosis and outcome, stratifying patients for different therapy regimens and tumor stages.