INRG Task Force

In 1988 an international task force was formed to standardize the risk group classification for neuroblastoma. The reason this is so important is because international studies could not be compared to each other with different patient cohorts. One “high risk” study might actually include children considered to be intermediate risk by another group, and the outcomes reported may consequently look “better.”

Today there are 64 investigators in the INRG task force which includes North America, Australia, Europe, and Japan. A consensus for a pre-treatment risk assignment has been accomplished, which includes image-defined risk factors (IDRFs) for staging. This believed to be more reproducible with radiologists.

A database of 8800 children diagnosed between 1990 and 2002 was used to characterize the new risk assignment scheme. In all of these children, the EFS was 63% ± 1% for all risk groups. The percentage of children in each risk group for the entire database breaks down to:

• 28% were very low risk
• 26% were low risk
• 9% were intermediate risk
• 36% were high risk

So far there have been many studies and reports using this data set.  Any investigator is invited to submit a bid for the use of the database analysis.

The most significant changes in risk assignment from this work is using IDRFs and including 11q status.

The challenge now is to incorporate and analyze approximately 4000 more children diagnosed since 2004 and added to the database.  This brings the total to over 16,000. New data items included are gender, ethnicity, therapy, and other causes of death.

A web-based interactive INRG database network has been proposed, and the goal is to collect biological data, phenotype, clinical outcome, and have tumor samples available for further analysis. A question for the future: will clinical features be abandoned and only genetic features used to classify risk assignment? This could allow for tweaking frontline therapy for children who have genetic aberrations predicting poor response to a particular therapy.

Clearly, this ambitious project will continue to offer a rich source of data to better predict outcomes and required therapy for children with neuroblastoma.

References:

Criteria for evaluation of disease extent by (123)I-metaiodobenzylguanidine scans in neuroblastoma: a report for the International Neuroblastoma Risk Group (INRG) Task Force. Matthay KK, Shulkin B, Ladenstein R, Michon J, Giammarile F, Lewington V, Pearson AD, Cohn SL. Br J Cancer. 2010 Apr 27;102(9):1319-26. Review.PMID: 20424613

International consensus for neuroblastoma molecular diagnostics: report from the International Neuroblastoma Risk Group (INRG) Biology Committee. Ambros PF, Ambros IM, Brodeur GM, Haber M, Khan J, Nakagawara A, Schleiermacher G, Speleman F, Spitz R, London WB, Cohn SL, Pearson AD, Maris JM. Br J Cancer. 2009 May 5;100(9):1471-82.PMID: 19401703

Consensus criteria for sensitive detection of minimal neuroblastoma cells in bone marrow, blood and stem cell preparations by immunocytology and QRT-PCR: recommendations by the International Neuroblastoma Risk Group Task Force. Beiske K, Burchill SA, Cheung IY, Hiyama E, Seeger RC, Cohn SL, Pearson AD, Matthay KK; International neuroblastoma Risk Group Task Force. Br J Cancer. 2009 May 19;100(10):1627-37. Epub 2009 Apr 28.PMID: 19401690

The International Neuroblastoma Risk Group (INRG) staging system: an INRG Task Force report. Monclair T, Brodeur GM, Ambros PF, Brisse HJ, Cecchetto G, Holmes K, Kaneko M, London WB, Matthay KK, Nuchtern JG, von Schweinitz D, Simon T, Cohn SL, Pearson AD; INRG Task Force. J Clin Oncol. 2009 Jan 10;27(2):298-303. Epub 2008 Dec 1.PMID: 19047290