Aggressive non-Hodgkin lymphoma in the pediatric and young adult population; diagnostic and molecular pearls of wisdom

Abstract

Mature non-Hodgkin lymphomas (NHLs) of the pediatric and young adults(PYA), including Burkitt lymphoma (BL), diffuse large B cell lymphoma (DLBCL), high-grade B cell lymphoma (HGBCL), primary mediastinal large B cell lymphoma (PMBL) and anaplastic large cell lymphoma (ALCL), generally have excellent prognosis compared to the adult population. BL, DLBCL and HGBCL are usually of germinal center (GCB) origin in the PYA population. PMBL neither belongs to the GCB nor the activated B cell subtype and is associated with a poorer outcome than BL or DLBCL of comparable stage. Anaplastic large cell lymphoma is the most frequent peripheral T cell lymphoma occurring in the PYA and accounts for 10–15% of childhood NHL. Most pediatric ALCL, unlike in the adult, demonstrate expression of anaplastic lymphoma kinase (ALK). In recent years, the understanding of the biology and molecular features of these aggressive lymphomas has increased tremendously. This has led to reclassification of newer PYA entities including Burkitt-like lymphoma with 11q aberration.

In this review, we will discuss the current progress discovered in frequently encountered aggressive NHLs in the PYA, highlighting the clinical, pathologic and molecular features that aid in the diagnosis of these aggressive lymphomas. We will be updating the new concepts and terminologies used in the new classification systems.