Primary Nonanaplastic Peripheral Natural Killer/T-Cell Lymphoma in Pediatric Patients-An Unusual Distribution Pattern of Subtypes.

ABSTRACT

Peripheral NK/T-cell lymphoma (PNKTCL) represents a group of uncommon diagnoses for children in Western countries, and studies have often necessitated multiple institutions to assemble enough cases. We retrospectively analyzed 11 cases of nonanaplastic PNKTCL in children over 19 years at our institution with comparison to several published large multi-institutional studies. Patients included 9 males and 2 females of white (5), Native American (3), and Hispanic (3) background with 6 cases of extranodal NK/T-cell lymphoma, nasal type (EN-NKTL, 54.6%), 3 cases of peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS, 27.2%), and 2 cases of systemic Epstein-Barr virus (EBV)-positive T-cell lymphoma of childhood (18.2%). Compared to the literature, our institution exceeded in frequency of total nonanaplastic PNKTCL (4.8% vs 0.9%-1.6%) with lesser relative incidence of PTCL-NOS (27.2% vs 42.9%-66.7%) and greater relative incidence of EN-NKTL (54.6% of cases vs 12.5%-47.6%), which significantly exceeded the literature's rate for Western institutions (13.5%). Potential influencing factors include population structure approximating those of non-Western countries with high EN-NKTL prevalence and the predisposition for EBV infection in this demography. These data suggest an uneven distribution of nonanaplastic PNKTCL in Western countries, and differential diagnoses may differ depending on practice location and associated patient population.