The International Neuroblastoma Staging System and International Neuroblastoma Risk Group
The first Forbeck Forum, held in 1985, focused on the topic ‘Neuroblastoma’, the rare pediatric tumor that had affected the Forbeck family and led to the establishing of the William Guy Forbeck Research Foundation. That Forum identified an important gap in the neuroblastoma field. Doctors in different clinics, in the United States and internationally, each had their own measures for monitoring neuroblastoma growth and response to treatment. A 1985 Forum recommendation was that clinics work together to develop consensus-based universal neuroblastoma monitoring criteria. By doing this, doctors could compare patient data between clinics for the first time. And because neuroblastoma is rare, and each clinic sees only a few cases, it provided an opportunity for different clinics to collaborate on research studies and clinical trials on patient populations. This process led to the creation of the International Neuroblastoma Staging System (INSS) – which have also been known as the ‘Forbeck Criteria’, in recognition of the Foundation’s key role in developing these. INSS also founded a group of international clinicians and scientists that has continued to expand and – with the Foundation’s support – meets periodically to review and update the INSS.
The International Neuroblastoma Risk Group (INRG) was formed in 1988 to standardize risk group classification for patients with neuroblastoma, addressing issues such as developing molecular markers to stage tumor growth and response to treatment. With over 60 members, this task force includes researchers from all over the world. The INRG created a database and standardized the risk factors for 9000 international patients, and in 2009, published a landmark paper in which a new risk assignment scheme was established for children with neuroblastoma. Over a dozen significant research papers have now been published using this important patient database.
The Interactive INRG Database
Researchers at the University of Chicago are developing an interactive website to allow investigators to query the INRG database, which now has over 10,000 international patients. Until now, researchers worked with statisticians to ask questions of the data and then had to ask other institutions worldwide whether or not patient samples were available. Now, for the first time, researchers wanting to study specific aspects of neuroblastoma will be able to directly query this rich database and also immediately know for which patients samples are available. This was established as the Interactive INRG database (INRGdb) in 2011. Centered at the University of Chicago, INRGdb has the support of the international INRG community. The investigators have reported that the database has gone through its first round of development and will be interfacing directly with the Children’s Oncology Group Tumor Bank in Columbus, Ohio. Data about this project were presented at the Advances in Neuroblastoma Research meeting in Toronto in June 2012, at which time a functioning demonstration was provided. The generous support of the Forbeck Foundation has allowed the project to hire a full-time Project Manager, who is directing the technical development under the guidance of Dr. Susan Cohn and Dr. Sam Volchenboum.
The INSS/INRG initiative has been an impressive achievement for WGFRF. Continuing to support this and expanding the database and continuing to support this effort overall are Foundation priorities for the future. Many key questions lie ahead. For example, despite the fact that the INSS neuroblastoma staging system has been taken up universally, the role of biological risk factors in staging the disease has never been fully addressed. It is anticipated that these and other questions will be addressed by the INRG in the future.