Survival and prognostic factors of early childhood medulloblastoma: An international meta-analysis

Stefan Rutkowski, Katja Von Hoff, Angela Emser, Isabella Zwiener, Torsten Pietsch, Dominique Figarella-Branger, Felice Giangaspero, David W. Ellison, Maria Luisa Garre, Veronica Biassoni, Richard G. Grundy, Jonathan L. Finlay, Girish Dhall, Marie Anne Raquin, Jacques Grill

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Abstract

Purpose: To assess the prognostic role of clinical parameters and histology in early childhood medulloblastoma. Patients and Methods: Clinical and histologic data from 270 children younger than age 5 years diagnosed with medulloblastoma between March 1987 and July 2004 and treated within prospective trials of five national study groups were centrally analyzed. Results: Two hundred sixty children with medulloblastoma and specified histologic subtype were eligible for analysis (median age, 1.89 years; median follow-up, 8.0 years). Rates for 8-year event-free survival (EFS) and overall survival (OS) were 55% and 76%, respectively, in 108 children with desmoplastic/nodular medulloblastoma (DNMB) or medulloblastoma with extensive nodularity (MBEN); 27% and 42%, respectively, in 145 children with classic medulloblastoma (CMB); and 14% and 14%, respectively, in seven children with large-cell/anaplastic (LC/A) medulloblastoma (P <.001). Histology (DNMB/MBEN: hazard ratio [HR], 0.44; 95% CI, 0.31 to 0.64; LC/A medulloblastoma: HR, 2.27; 95% CI, 0.95 to 5.54; P <.001 compared with CMB), incomplete resection and metastases (M0R1: HR, 1.86; 95% CI, 1.29 to 2.80; M+: HR, 2.28; 95% CI, 1.50 to 3.46; P <.001 compared with M0R0), and national group were independent prognostic factors for EFS, and OS. The HRs for OS ranged from 0.14 for localized M0 and DNMB/MBEN to 13.67 for metastatic LC/A medulloblastoma in different national groups. Conclusion: Our results confirm the high frequency of desmoplastic variants of medulloblastomas in early childhood and histopathology as a strong independent prognostic factor. A controlled de-escalation of treatment may be appropriate for young children with DNMB and MBEN in future clinical trials.

Original languageEnglish
Pages (from-to)4961-4968
Number of pages8
JournalJournal of Clinical Oncology
Volume28
Issue number33
DOIs
Publication statusPublished - Nov 20 2010

ASJC Scopus subject areas

  • Cancer Research
  • Oncology

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    Rutkowski, S., Von Hoff, K., Emser, A., Zwiener, I., Pietsch, T., Figarella-Branger, D., Giangaspero, F., Ellison, D. W., Garre, M. L., Biassoni, V., Grundy, R. G., Finlay, J. L., Dhall, G., Raquin, M. A., & Grill, J. (2010). Survival and prognostic factors of early childhood medulloblastoma: An international meta-analysis. Journal of Clinical Oncology, 28(33), 4961-4968. https://doi.org/10.1200/JCO.2010.30.2299