An international coalition of pediatric cancer physicians and researchers has developed new systems to standardize studies of neuroblastomas across the world. In the December issue of the Journal of Clinical Oncology, the International Neuroblastoma Risk Group (INRG) presents three sets of papers outlining a: standard classification system; pre-treatment staging system; and an analysis of a rare group of patients.
The INRG studies provide for a unified system of clinical trials that will enable quicker identification of optimal treatments for neuroblastoma.

Neuroblastoma is the most common solid cancer that occurs outside the cranium during childhood. For some young children, it disappears with minimal treatment. In other children, it can be relentlessly aggressive, with a high likelihood of death. Predicting the behavior of this tumor is crucial in planning appropriate treatment.
The INRG task force is co-chaired by Susan Cohn, professor and director of clinical sciences at the University of Chicago Comer Children’s Hospital, and Andrew Pearson, chairman of paediatric oncology at the Institute of Cancer Research at Royal Marsden Hospital in the UK.

Cohn says that in the past, criteria used to predict tumor behavior and stratify treatment have not been uniform throughout the world, which makes it impossible to directly compare clinical trial results. The INRG classification system is designed to create consistency of risk group assignment around the globe, and will facilitate clinical research.

“We strongly recommend that cooperative groups begin using this classification system now,” Cohn says.

“The system will allow the direct comparison of results from clinical trials conducted in different regions of the world and will help us determine the best treatment strategies for patients with neuroblastoma,” she adds. “By working together, physicians will be able to ask questions about treatment approaches that would otherwise not be possible to ask in a single cooperative group or country because of the small numbers of patients. We plan to continue to expand this database, and as new molecular tools are developed to test cancer genetics, the INRG Classification System will be refined.”

Formed in 2004, the INRG task force has 52 members working to develop a standard worldwide approach to classify neuroblastomas before treatment.
The criteria included in the INRG classification system were based on an analysis of 8,800 children under age 21 who were diagnosed with neuroblastoma between 1990-2002. They were primarily from North America, Australia, Europe, and Japan. Among the seven predictors of how the neuroblastoma will progress are stage of the cancer as defined by INRG standards, histology, patient’s age, and genetic factors.

The INRG database has also proven to be an invaluable resource for additional research. The third paper in the issue of the Journal of Clinical Oncology describes an analysis of a very rare subset of patients with low-stage disease and amplification of the MYCN oncogene.

The study was funded, in part, by the William Guy Forbeck Research Foundation and the Little Heroes Pediatric Cancer Research Foundation.

Additional study authors for the three studies include Wendy London, Tom Monclair, Peter Ambros, Garrett Brodeur, Andreas Faldum, Barbara Hero, Tomoko Iehara, David Machin, Veronique Mosseri, Thorsten Simon, Victoria Castel, Katherine Matthay, Keith Holmes, Alberto Garaventa, Giovanni Cecchetto, Herve Brisse, Michio Kaneko, Jed Nuchtern, Dietrich von Schweinitz, Rochelle Bagatell, Maja Beck-Popovic, and Yang Zhang.

Source: University of Chicago Medical Center