Rhabdoid tumors in children: Prognostic factors in 70 patients diagnosed in Germany

Reinhard,Harald; Reinert,Jennifer; Beier,Rita; Furtwängler,Rhoikos; Alkasser,Muhanat; Rutkowski,Stefan; Frühwald,Michael; Koscielniak,Ewa; Leuschner,Ivo; Kaatsch,Peter; Graf,Norbert

doi:10.3892/or.19.3.819

March 2008 Volume 19 Issue 3

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March 2008 Volume 19 Issue 3

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Article

Rhabdoid tumors in children: Prognostic factors in 70 patients diagnosed in Germany

Authors:
- Harald Reinhard
- Jennifer Reinert
- Rita Beier
- Rhoikos Furtwängler
- Muhanat Alkasser
- Stefan Rutkowski
- Michael Frühwald
- Ewa Koscielniak
- Ivo Leuschner
- Peter Kaatsch
- Norbert Graf
View Affiliations / Copyright

Affiliations: Pediatric Hematology and Oncology, University Hospital, D-66421 Homburg/Saar, Germany. harald.reinhard@uniklinikum-saarland.de
Pages: 819-823
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Published online on: March 1, 2008

https://doi.org/10.3892/or.19.3.819
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Abstract

We retrospectively analysed the data of 70 patients (41 boys and 29 girls) with Rhabdoid tumors (RT) regardless of localisation, recorded in the German Childhood Cancer Registry (GCCR) from 1984 to 1999. The primary tumor was located in the kidney in 32 cases, in the central nervous system (CNS) in 13 cases and in the soft tissue in 25 cases. Variables examined were tumor stage, sex, age at diagnosis, surgical radicality, radiotherapy and chemotherapeutic regimens. Metastatic disease at diagnosis was observed in 18 of the 70 individuals. Outcome of this group was very poor with a 5-year overall survival of 11%. There were no differences in survival between males and females, or younger and older children. Chemotherapeutic regimens were mainly given according to the primary site of the tumor. Radiotherapy was given in 28 of the 70 patients with a mean dose of 35 Gray, though this did not improve the outcome. Overall survival of the whole cohort was 27% at 5 years and there was no significant difference in prognosis regarding the different locations of the tumor (kidney 24%, soft tissue 30%, CNS 29%). In conclusion, RT in infants and children has a dismal prognosis, independent from localisation. The presence of metastasis at diagnosis seems to be the only prognostic factor of outcome.

Journals

International Journal of Molecular Medicine

International Journal of Oncology

Molecular Medicine Reports

Oncology Reports

Experimental and Therapeutic Medicine

Oncology Letters

Biomedical Reports

Molecular and Clinical Oncology

World Academy of Sciences Journal

International Journal of Functional Nutrition

International Journal of Epigenetics

Medicine International

Rhabdoid tumors in children: Prognostic factors in 70 patients diagnosed in Germany

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Abstract

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