Conflict of interest: Nothing to report.
Rare malignant pediatric tumors registered in the German Childhood Cancer Registry 2001–2010
Version of Record online: 28 FEB 2014
© 2014 Wiley Periodicals, Inc.
Pediatric Blood & Cancer
Volume 61, Issue 7, pages 1202–1209, July 2014
How to Cite
Brecht, I. B., Bremensdorfer, C., Schneider, D. T., Frühwald, M. C., Offenmüller, S., Mertens, R., Vorwerk, P., Koscielniak, E., Bielack, S. S., Benesch, M., Hero, B., Graf, N., ∨on Schweinitz, D. and Kaatsch, P. (2014), Rare malignant pediatric tumors registered in the German Childhood Cancer Registry 2001–2010. Pediatr. Blood Cancer, 61: 1202–1209. doi: 10.1002/pbc.24997
- Issue online: 2 MAY 2014
- Version of Record online: 28 FEB 2014
- Manuscript Accepted: 28 JAN 2014
- Manuscript Received: 24 JUN 2013
- German Childhood Cancer Foundation (“Deutsche Kinderkrebsstiftung”), Bonn, Germany
- “Verein zur Förderung des Tumorzentrums Erlangen”, Erlangen, Germany
- cancer registry;
- orphan disease;
- pediatric oncology;
- rare tumors;
The German Childhood Cancer Registry (GCCR) annually registers approximately 2,000 children diagnosed with a malignant disease (completeness of registration >95%). While most pediatric cancer patients are diagnosed and treated according to standardized cooperative protocols of the German Society for Pediatric Oncology and Hematology (GPOH), patients with rare tumors are at risk of not being integrated in the network including trials and reference centers.
A retrospective analysis of all rare extracranial solid tumors reported to the GCCR 2001–2010 (age <18 years) was undertaken using a combination of the International Classification of Childhood Cancer (ICCC-3) and the International Classification of Diseases-Oncology (ICD-O-3). Tumors accounting for <0.3% of all malignancies were defined as rare (approx. 6 cases/year and registered malignancy).
According to this definition 1,189 rare extracranial solid tumors (18.2% of all malignant extracranial solid tumors) were registered, among these 232 patients (19.5% of rare tumor cases), were not included in preexisting GPOH studies/registries. Within 10 years, the number of registered non-GPOH-trial patients with a rare tumor increased.
Though most of the GCCR-registered patients with rare malignant tumors are treated within GPOH trials, there is a considerable number of patients that have been diagnosed and treated outside the structures of the GPOH. These patients should be reported to the recently founded German Pediatric Rare Tumor Registry (STEP). Active data accrual and the development of appropriate structures will allow for better registration and improvement of medical care in these patients. Pediatr Blood Cancer 2014;61:1202–1209. © 2014 Wiley Periodicals, Inc.