A Survival Analysis of Pediatric Tonsillar Malignancies and Review of a Rare Case

Objective: To examine the survival of pediatric tonsillar cancer patients and review a rare case of pediatric tonsillar cancer. Methods: Pediatric patients in the Surveillance, Epidemiology, and End Results (SEER) database were included from 1973 to 2014 based on a diagnosis of tonsillar malignancy using the ICD O-3 tonsil primary site codes of: C09.0, C09.1, C09.8, and C09.9. Patients were included from birth-18 years. Survival analysis was performed using Kaplan-Meier analysis. Additionally, a case of pediatric natural killer (NK) cell tonsillar lymphoma diagnosed and treated at the Nemours Children's hospital in Orlando, Florida is presented. Results: One hundred forty-one cases of tonsil cancer were identified. The mean age at diagnosis was 9.9 years (SD: 5.1, range: 0.0 (months)–18.0). Ninety five (67.4%) patients were male and 116 (82.3%) had unilateral malignancies. Burkitt lymphoma (32.6%) followed by diffuse large B-cell lymphoma (DLBCL) (27.0%) were the two most common histological types of tonsillar cancers. 79.4% of patients received chemotherapy and 81.6% received surgery as a part of their care. The 5-year disease-specific survival rate was >90% for patient cohorts diagnosed from 1984 to 1993, 1994–2003, and 2004–2014 as compared to 64% for patients diagnosed from 1973 to 1983 (p = 0.01). Conclusions: Survival rates for pediatric patients with tonsillar cancer are excellent. Pediatric primary tonsil cancer occurred most commonly in adolescent males and usually presents as a unilateral mass. Lymphoma remains the predominant histological type of cancer. Most patients are likely to receive surgery and chemotherapy.