Revised International Pediatric Non-Hodgkin Lymphoma Staging System

Abstract

Purpose: Treatment and prognosis of pediatric non-Hodgkin lymphoma (NHL) have improved dramatically in the last 30 years. However, the St Jude NHL staging classification for pediatric NHL was developed more than 35 years ago. The most recent Lugano lymphoma staging classification focused on adult lymphoma. Furthermore, major limitations of the current pediatric NHL staging classification include lack of consideration of new distinct pediatric NHL histologic entities; absence of recognition of frequent skin, bone, kidney, ovarian, and other organ involvement; and lack of newer precise methods to detect bone marrow and CNS involvement, minimal disease quantification, and highly sensitive imaging technologies.

Methods: An international multidisciplinary expert panel convened in Frankfurt, Germany, in 2009 at the Third International Childhood, Adolescent and Young Adult NHL Symposium to develop a revised international pediatric NHL staging system (IPNHLSS), addressing limitations of the current pediatric NHL staging system and creating a revised classification. Evidence-based disease distribution and behavior were reviewed from multiple pediatric cooperative group NHL studies.

Results: A revised IPNHLSS was developed incorporating new histologic entities, extranodal dissemination, improved diagnostic methods, and advanced imaging technology.

Conclusion: This revised IPNHLSS will facilitate more precise staging for children and adolescents with NHL and facilitate comparisons of efficacy across different treatment strategies, various institutions, multicenter trials, and cooperative groups by allowing for reproducible pediatric-based staging at diagnosis and relapse.

Conflict of interest statement

Authors' disclosures of potential conflicts of interest are found in the article online at www.jco.org. Author contributions are found at the end of this article.

© 2015 by American Society of Clinical Oncology.

Figures

Fig 1.

Survival analysis. Progression-free survival (PFS) analysis (A) in patients with Burkitt's lymphoma at high risk (Berlin-Frankfurt-Munster high-risk group R4) according to morphologic bone marrow (BM) status at diagnosis and (B) according to minimal disseminated disease (MDD) status in BM at diagnosis. Data adapted.

Fig 2.

Event-free survival according to levels of T-cell lymphoblastic lymphoma (T-LL) cells in bone marrow at diagnosis measured by flow cytometry. Data adapted.

Fig 3.

Outcome of patients with anaplastic large-cell lymphoma according to quantitative polymerase chain reaction (PCR) results for NPM-ALK in bone marrow. (A) Cumulative incidence of relapse and (B) Kaplan-Meier estimates of event-free survival of 74 patients with either negative qualitative bone marrow (BM) PCR or positive qualitative BM PCR using quantitative (quant) PCR results and NPM-ALK cutoff copy number of 10 of 104 copies of ABL (normalized copy number). NR, nonrelapse. Data adapted.