Originally published as JCO Early Release 10.1200/JCO.2008.16.6876 on December 1 2008

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The International Neuroblastoma Risk Group (INRG) Staging System: An INRG Task Force Report

Tom Monclair, Garrett M. Brodeur, Peter F. Ambros, Hervé J. Brisse, Giovanni Cecchetto, Keith Holmes, Michio Kaneko, Wendy B. London, Katherine K. Matthay, Jed G. Nuchtern, Dietrich von Schweinitz, Thorsten Simon, Susan L. Cohn, Andrew D.J. Pearson

From the Section for Paediatric Surgery, Division of Surgery, Rikshospitalet University Hospital, Oslo, Norway; Division of Oncology, Children's Hospital of Philadelphia, Philadelphia, PA; Children's Cancer Research Institute, St Anna Kinderkrebsforschung, Vienna, Austria; Department of Radiology, Institut Curie, Paris, France; Pediatric Surgery-Department of Pediatrics, University of Padova, Padova, Italy; Department of Paediatric Surgery, St George's Hospital, London; Section of Paediatrics, Institute of Cancer Research and Royal Marsden Hospital, Surrey, United Kingdom; Department of Pediatric Surgery, University of Tsukuba, Tsukuba, Japan; Children's Oncology Group and Department of Epidemiology and Health Policy Research, University of Florida, Gainesville, FL; Department of Pediatrics, University of California School of Medicine, San Francisco, CA; Department of Pediatric Surgery, Texas Children's Hospital, Houston, TX; Department of Pediatric Surgery, Dr. von Hauner'sches Kinderspital, University of Munich, Munich; Department of Pediatric Oncology and Hematology, Children's Hospital, University of Cologne, Germany; and Department of Pediatrics, the University of Chicago, Chicago, IL

Corresponding author: Tom Monclair, MD, PhD, Section for Paediatric Surgery, Division of Surgery, Rikshospitalet University Hospital, NO-0027 Oslo, Norway; e-mail: tom.monclair{at}rikshospitalet.no

Purpose The International Neuroblastoma Risk Group (INRG) classification system was developed to establish a consensus approach for pretreatment risk stratification. Because the International Neuroblastoma Staging System (INSS) is a postsurgical staging system, a new clinical staging system was required for the INRG pretreatment risk classification system.

Methods To stage patients before any treatment, the INRG Task Force, consisting of neuroblastoma experts from Australia/New Zealand, China, Europe, Japan, and North America, developed a new INRG staging system (INRGSS) based on clinical criteria and image-defined risk factors (IDRFs). To investigate the impact of IDRFs on outcome, survival analyses were performed on 661 European patients with INSS stages 1, 2, or 3 disease for whom IDRFs were known.

Results In the INGRSS, locoregional tumors are staged L1 or L2 based on the absence or presence of one or more of 20 IDRFs, respectively. Metastatic tumors are defined as stage M, except for stage MS, in which metastases are confined to the skin, liver, and/or bone marrow in children younger than 18 months of age. Within the 661-patient cohort, IDRFs were present (ie, stage L2) in 21% of patients with stage 1, 45% of patients with stage 2, and 94% of patients with stage 3 disease. Patients with INRGSS stage L2 disease had significantly lower 5-year event-free survival than those with INRGSS stage L1 disease (78% ± 4% v 90% ± 3%; P = .0010).

Conclusion Use of the new staging (INRGSS) and risk classification (INRG) of neuroblastoma will greatly facilitate the comparison of risk-based clinical trials conducted in different regions of the world.

published online ahead of print at www.jco.org on December 1, 2008.

Supported by the William Guy Forbeck Research Foundation and Little Heroes Pediatric Cancer Research Fund; and Cancer Research UK and NHS funding to the NIHR Biomedical Research Centre (to A.D.J.P.).

Presented in part at the Advances in Neuroblastoma Research 12th Conference, May 17-20, 2006, Los Angeles, CA, and the International Society of Paediatric Oncology 38th Congress, September 18-21, 2006, Geneva, Switzerland.

Authors’ disclosures of potential conflicts of interest and author contributions are found at the end of this article.

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