Pediatric sporadic Burkitt lymphoma of the head and neck: A case series and analysis of national trends

Objective: Burkitt lymphoma (BL) is an aggressive form of non-Hodgkin lymphoma with the sporadic subtype being predominant in North America. The clinical presentations and outcomes of pediatric BL within the head and neck were assessed using both an institutional case series and the Surveillance, Epidemiology, and End Results (SEER) Cancer database. Methods: The electronic medical record at our quaternary children's hospital was queried over a 22-year period (2000-2022) for BL patients with head and neck manifestations. Demographics, clinical presentation, staging, treatment, and outcomes data were collected and analyzed. A corresponding review of the SEER database from 1975 to 2022 was also performed. Results: Our institutional case series identified 48 sporadic BL patients with a mean age of 8.7 years, the majority of whom were male (79 %) and white (74 %). The most common primary sites were the cervical lymph nodes (38 %) and (or) palatine tonsils (23 %). Thirty-five patients (73 %) were treated initially for a presumed inflammatory or infectious process before undergoing malignancy work-up, which did not significantly delay time to diagnosis (31.5 vs. 38.8 days, p = 0.27). The SEER database analysis identified 78 cases, 43.5 % of whom were 5-9 years of age, with a similar male (66 %) and Caucasian (76.9 %) predominance. Cervical lymph nodes were also the most common subsite (67 %), followed by the palatine tonsils (13 %). Remission rates were similar, 93.7 % and 94.8 %, respectively, in both the institutional and SEER database cohorts. Conclusion: Unilateral cervical lymphadenopathy and asymmetric tonsillar hypertrophy are the most common presentations in sporadic BL in the head and neck. Clinical presentation in patients with BL is often similar to common, insidious pediatric otolaryngology symptoms and a majority of patients initially undergo treatment for presumed infectious or inflammatory disease. Although overall BL disease-free survival is high even for disseminated BL, the prognosis is better for local/regional disease, and minimizing time to diagnosis and treatment should remain a priority.