Gerardo Petruzzi1, Andrea Costantino2,3, Giuseppe Spriano2,3, Raul Pellini1, Armando De Virgilio4,5, Jacopo Zocchi1, Flaminia Campo1, Giuseppe Mercante2,3. 1. Department of Otolaryngology and Head and Neck Surgery, IRCCS Regina Elena National Cancer Institute, Rome, Italy. 2. Department of Biomedical Sciences, Humanitas University, Via Rita Levi Montalcini 4, Pieve Emanuele, 20090, Milan, Italy. 3. Otorhinolaryngology Unit, IRCCS Humanitas Research Hospital, Via Manzoni 56, Rozzano, 20089, Milan, Italy. 4. Department of Biomedical Sciences, Humanitas University, Via Rita Levi Montalcini 4, Pieve Emanuele, 20090, Milan, Italy. armando.devirgilio@gmail.com. 5. Otorhinolaryngology Unit, IRCCS Humanitas Research Hospital, Via Manzoni 56, Rozzano, 20089, Milan, Italy. armando.devirgilio@gmail.com.
Abstract
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.
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.