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Klin Padiatr 2012; 224(06): 416-420
DOI: 10.1055/s-0032-1327608
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© Georg Thieme Verlag KG Stuttgart · New York

Rare Cancers in Children – The EXPeRT Initiative: A Report from the European Cooperative Study Group on Pediatric Rare Tumors

Seltene Tumoren bei Kindern – die EXPeRT Initiative: Ein Bericht der European Cooperative Study Group on Pediatric Rare Tumors
G. Bisogno   *
1   Clinic of Pediatric Oncology, University of Padova, Padova, Italy
,
A. Ferrari   *
2   Pediatric Oncology, Fondazione IRCCS Istituto Nazionale Tumori, Milan, Italy
,
E. Bien
3   Department of Pediatrics, Hematology, Oncology and Endocrinology, Medical University of Gdansk, Gdansk, Poland
,
I. B. Brecht
4   Pediatric Hematology and Oncology, University Children’s Hospital ­Erlangen, Erlangen, Germany
,
B. Brennan
5   Pediatric Oncology, Royal Manchester Children’s Hospital, Manchester, United Kingdom
,
G. Cecchetto
6   Pediatric Surgery, University of Padova, Padova, Italy
,
J. Godzinski
7   Department of Pediatric Surgery, Marciniak Hospital, Wroclaw, Poland
,
D. Orbach
8   Pediatric Hematology and Oncology, Institute Gustave Curie, Paris, France
,
Y. Reguerre
9   Pediatric Hematology and Oncology, University Hospital Angers, Angers, France
,
T. Stachowicz-Stencel
3   Department of Pediatrics, Hematology, Oncology and Endocrinology, Medical University of Gdansk, Gdansk, Poland
,
D. T. Schneider   *
10   Klinik für Kinder- und Jugendmedizin, Klinikum Dortmund gGmbH, ­Dortmund, Germany
› Author Affiliations
Further Information

Publication History

Publication Date:
09 November 2012 (online)

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Abstract

The low incidence and the heterogeneity of very rare tumors (VRTs) demand for international cooperation. In 2008, EXPeRT (European Cooperative Study Group for Pediatric Rare Tumors) was founded by national groups from Italy, France, United Kingdom, Poland and Germany. The first aims of EXPeRT were to agree on a uniform definition of VRTs and to develop the currently most relevant scientific questions. Current initiatives include international data exchange, retrospective and prospective studies of specific entities, and the development of harmonized and internationally recognized guidelines. Moreover, EXPeRT established a network for expert consultation to assist in clinical decision in VRTs.

Zusammenfassung

Seltene Tumoren zeichnen sich nicht nur durch eine niedrige Inzidenz sondern auch durch ihre ausgeprägte eine klinische und histologische Heterogenität aus. Um die Forschungsaktivitäten auf diesem Gebiet zu unterstützen, wurde 2008 eine internationale Arbeitsgruppe, die European Cooperative Study Group on Pediatric Rare Tumors (EXPeRT), gegründet. In ihr kooperie­ren die Arbeitsgruppen aus Italien, Frankreich, Großbritannien, Polen und Deutschland. Als erstes Ziel wurden eine einheitliche Definition der Seltenen Tumoren entwickelt und wissenschaftliche Fragestellungen formuliert. Aktuelle Initiativen beinhalten den Datenaustausch und gemeinsame retro- und prospektive Analysen für bestimmte Entitäten. Darüber hinaus wird eine Vereinheitlichung der Behandlungsempfehlungen für Seltene Tumoren angestrebt. Auf dieser Grundlage baut EXPeRT ein klinisches und wissenschaftliches Konsiliarnetz auf, das anfragende Kliniken bei anstehenden klinischen Entscheidungen bei Kindern und Jugendlichen mit seltenen Tumoren unterstützt.

Key words

rare tumors - epidemiology - children - network - orphan disease

Schlüsselwörter

seltene Tumoren - Epidemiologie - Netzwerk - Register
*

* These authors contributed equally to this manuscript.


 

  • References

  • 1 Balcerska A, Godzinski J, Bien E et al. Rare tumours – are they really rare in the Polish children population?. Przegl Lek 2004; 61: 57-61
    PubMedGoogle Scholar
  • 2 Benesch M, Leuschner I, Wardelmann E et al. Gastrointestinal stromal tumours in children and young adults: a clinicopathologic series with long-term follow-up from the database of the Cooperative Weichteilsarkom Studiengruppe (CWS). Eur J Cancer 2011; 47: 1692-1698
    CrossrefPubMedGoogle Scholar
  • 3 Bien E, Godzinski J, Dall’igna P et al. Pancreatoblastoma: a report from the European cooperative study group for paediatric rare tumours (EXPeRT). Eur J Cancer 2011; 47: 2347-2352
    CrossrefPubMedGoogle Scholar
  • 4 Brecht IB, Graf N, Schweinitz D et al. Networking for children and adolescents with very rare tumors: foundation of the GPOH Pediatric Rare Tumor Group. Klin Pädiatr 2009; 221: 181-185
    Article in Thieme ConnectPubMedGoogle Scholar
  • 5 Brecht IB, Schneider DT, Klöppel G et al. Malignant pancreatic tumors in children and young adults: evaluation of 228 patients identified through the Surveillance, Epidemiology, and End Result (SEER) database. Klin Pädiatr 2011; 223: 341-345
    Article in Thieme ConnectPubMedGoogle Scholar
  • 6 Brennan B. Pediatric pancreatic tumors: the orphan looking for a home. Pediatr Blood Cancer 2010; 54: 659-660
    PubMedGoogle Scholar
  • 7 Ferrari A, Bisogno G, De Salvo GL et al. The challenge of very rare tumours in childhood: the Italian TREP project. Eur J Cancer 2007; 43: 654-659
    CrossrefPubMedGoogle Scholar
  • 8 Ferrari A, Sultan I. When adult cancers occur in children. Expert Rev Anticancer Ther 2010; 10: 1683-1685
    CrossrefPubMedGoogle Scholar
  • 9 French CA. NUT midline carcinoma. Cancer Genet Cytogenet 2010; 203: 16-20
    CrossrefPubMedGoogle Scholar
  • 10 Gatta G, Ferrari A, Stiller CA et al. Embryonal cancers in Europe. Eur J Cancer 2012; 48: 1425-1433
    CrossrefPubMedGoogle Scholar
  • 11 Göbel U, Gortner L. Disease management programs for adults with often diseases and competence networks for children and adolescents with rare diseases. Klin Pädiatr 2011; 223: 1-3
    Article in Thieme ConnectPubMedGoogle Scholar
  • 12 Hill DA, Ivanovich J, Priest JR et al. DICER1 mutations in familial pleuropulmonary blastoma. Science 2009; 325: 965
    CrossrefPubMedGoogle Scholar
  • 13 Lacour B, Guyot-Goubin A, Guissou S et al. Incidence of childhood cancer in France: National Children Cancer Registries, 2000-2004. Eur J Cancer Prev 2010; 19: 173-181
    CrossrefPubMedGoogle Scholar
  • 14 Merks HHM, Brecht IB. Genetic Predisposition and Genetic Susceptibility. In: Schneider DT, Brecht IB, Olson TA, Ferrari A. (eds.). Rare Tumors In Children and Adolescents. Springer; 2012. 69-96
    CrossrefGoogle Scholar
  • 15 Olson TA, Schneider DT, Brecht IB et al. Rare Tumors: A Different Perspective on Oncology. In: Schneider DT, Brecht IB, Olson TA, Ferrari A. (eds.). Rare Tumors In Children and Adolescents. Springer; 2012. 3-15
    CrossrefGoogle Scholar
  • 16 Orbach D, Reguerre Y, Brecht IB et al. National and International Groups. In: Schneider DT, Brecht IB, Olson TA, Ferrari A. (eds.). Rare Tumors In Children and Adolescents. Springer; 2012. 97-120
    CrossrefGoogle Scholar
  • 17 Pappo AS, Krailo M, Chen Z et al. Infrequent tumor initiative of the Children’s Oncology Group: initial lessons learned and their impact on future plans. J Clin Oncol 2010; 28: 5011-5016
    CrossrefPubMedGoogle Scholar
  • 18 Pastore G, De Salvo GL, Bisogno G et al. Evaluating access to pediatric cancer care centers of children and adolescents with rare tumors in Italy: the TREP project. Pediatr Blood Cancer 2009; 53: 152-155
    CrossrefPubMedGoogle Scholar
  • 19 Priest JR, Watterson J, Strong L et al. Pleuropulmonary blastoma: a marker for familial disease. JPediatr 1996; 128: 220-224
    CrossrefPubMedGoogle Scholar
  • 20 Reguerre Y, Lacour B, Andre N et al. Epidemiology and management of rare paediatric tumours within the framework of the French Society for Children Cancer. Bull Cancer 2010; 97: 1041-1045
    PubMedGoogle Scholar
  • 21 Rodriguez-Galindo C, Figueiredo BC, Zambetti GP et al. Biology, clinical characteristics, and management of adrenocortical tumors in children. Pediatr Blood Cancer 2005; 45: 265-273
    CrossrefPubMedGoogle Scholar
  • 22 Sameshima Y, Tsunematsu Y, Watanabe S et al. Detection of novel germ-line p53 mutations in diverse-cancer-prone families identified by selecting patients with childhood adrenocortical carcinoma. J Natl Cancer Inst 1992; 84: 703-707
    CrossrefPubMedGoogle Scholar
  • 23 Schneider DT, Brecht IB. Care for rare cancers: improved care requires improved communication. Klin Pädiatr 2010; 222: 124-126
    Article in Thieme ConnectPubMedGoogle Scholar
  • 24 Schneider DT, Orbach D, Cecchetto G et al. Sertoli-Leydig Cell Tumors: An Analysis of the European Cooperative Study Group on Pediatric Rare Tumors (eXpert). Pediatric Blood Cancer. 2010 55
    PubMedGoogle Scholar
  • 25 Stachowicz-Stencel T, Bien E, Balcerska A et al. Diagnosis and treatment of renal cell carcinoma in children: a report from the Polish pediatric rare tumor study group. Klin Pädiatr 2011; 223: 138-141
    Article in Thieme ConnectPubMedGoogle Scholar
  • 26 Wagner J, Portwine C, Rabin K et al. High frequency of germline p53 mutations in childhood adrenocortical cancer. J Natl Cancer Inst 1994; 86: 1707-1710
    CrossrefPubMedGoogle Scholar