Young and naïve B cells are a diagnostic pitfall in pediatric tonsillectomies

Tonsillar marginal zone hyperplasia may mimic mucosa-associated lymphoid tissue lymphoma, a rare diagnosis in children. Histologically, both entities can demonstrate expansion of the marginal zone with disruption of follicular architecture. However, marginal zone hyperplasia may appear polyclonal by flow cytometry. We present two pediatric tonsillectomy cases with tonsillar marginal zone hyperplasia and discuss the diagnostic challenges this poses in the pediatric population. Both tonsillectomies demonstrated expansion of marginal zones with partial architectural effacement, and flow cytometric analysis of both cases detected lambda light chain restricted, CD20(bright) B cells without CD38. Authors have suggested that the lambda restricted B cells in this setting represent naïve, unmutated B cells with preferential, but polyclonal, lambda expression. Our cases are in line with this thought. While robust, BIOMED-2 primer PCR can show dominant IgK peaks, which may be misinterpreted. This presents a diagnostic pitfall in the workup of pediatric tonsils that community pathologists must consider.