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Supratentorial primitive neuroectodermal tumors: a Canadian pediatric brain tumor consortium report
Donna L Johnston1, Daniel L Keene, Lucie Lafay-Cousin
1Division of Hematology/Oncology, Children's Hospital of Eastern Ontario, 401 Smyth Road, Ottawa, ON, Canada, K1H 8L1. djohnston@cheo.on.ca
Introduction:
Supratentorial primitive neuroectodermal tumors (SPNET) are rare tumors accounting for only 2.5% of childhood brain tumors. The purpose of this study was to describe the range of treatment regimens used to treat pediatric SPNET in Canada and to identify prognostic factors for overall survival in this population.
Methods:
This study was a retrospective clinical analysis of SPNET patients treated over the last 10 years in Canada. A questionnaire was developed and distributed to all institutions in Canada who treat pediatric patients. Data were collected for patients <19 years of age who were diagnosed and treated for SPNET between 1995 and 2005.
Results:
Data were obtained for 48 eligible patients. The stages of patients for whom complete data were provided were 80, 3, and 16% for metastatic stage M0, M1, and M2/3, respectively. The best responses to therapy included complete response in 44%, partial response in 8%, still on therapy in 2%, progressive disease in 31%, toxic death in 2%, and no therapy given in 12%. The 4-year survival was 37.7 +/- 7.6%. The factors associated with an increase in survival were the use of radiation therapy and chemotherapy, and age >2 years. Overall survival was not affected by metastatic disease at diagnosis, tumor site, or degree of initial resection.
Conclusions:
Survival is poor in SPNET patients but highest in those who received chemotherapy and radiation therapy. Further studies are needed to improve the survival of these patients.
Insights
Treatment with chemotherapy and radiation therapy offers the best survival for pediatric supratentorial primitive neuroectodermal tumors (SPNET). Further research is needed to improve outcomes for these rare childhood brain tumors.
Area of Science:
- Pediatric Oncology
- Neuro-oncology
- Cancer Research
Background:
- Supratentorial primitive neuroectodermal tumors (SPNET) are rare, comprising only 2.5% of childhood brain tumors.
- Understanding treatment patterns and survival factors is crucial for this vulnerable population.
Purpose of the Study:
- To analyze treatment regimens for pediatric SPNET in Canada.
- To identify prognostic factors influencing overall survival in pediatric SPNET patients.
Main Methods:
- Retrospective clinical analysis of SPNET patients treated in Canada (1995-2005).
- Data collected via questionnaire distributed to pediatric treatment institutions.
- Analysis included 48 eligible patients under 19 years of age.
Main Results:
- Four-year survival rate was 37.7%.
- Chemotherapy and radiation therapy significantly increased survival.
- Age over 2 years was associated with improved survival; metastatic disease, tumor site, and resection degree did not significantly impact survival.
Conclusions:
- Pediatric SPNET patients have poor survival rates.
- Combined chemotherapy and radiation therapy yield the highest survival.
- Further studies are essential to enhance treatment efficacy and survival outcomes.
