Paediatric-Type Follicular Lymphoma: A Distinct Clinicopathologic Entity in Children and Adolescents. A short reviewa

Abstract Pediatric-type follicular lymphoma (PTFL) is a rare, indolent B-cell neoplasm accounting for <2% of all pediatric non-Hodgkin lymphomas. Recently recognized as a distinct entity, PTFL predominantly affects young males and typically presents as localized lymphadenopathy in the head and neck region. Histologically, PTFL shows nodal effacement by large, irregular follicles composed of intermediate-sized blastoid cells with germinal center phenotype. On a genetic level, PTFL lacks BCL2 rearrangements and shows recurrent mutations of MAP2K1 and TNFRSF14 genes. The disease has an excellent prognosis, with 5-year overall survival over 95% and extremely low progression rates. The management is usually conservative after complete surgical excision. In this review, we highlight the unique clinical-pathological and biological features of PTFL, with a focus on its differential diagnosis and on potential therapeutic approaches.