Survival Rates and Prognostic Factors in Childhood Medulloblastoma and Supratentorial Primitive Neuroectodermal Tumor.
- Author:
Pil Sang JANG
1
;
Keon Hee YOO
;
Dong Soon LEE
;
Chong Jai KIM
;
Hee Young SHIN
;
Kyu Chang WANG
;
Il Han KIM
;
In One KIM
;
Hyo Seop AHN
Author Information
1. Department of Pediatrics, Seoul National University, College of Medicine, Seoul, Korea. hyshin@plaza.snu.ac.kr
- Publication Type:Original Article
- Keywords:
Medulloblastoma;
Supratentorial primitive neuroectodermal tumor;
Prognostic factor;
Survival
- MeSH:
Child;
Child, Preschool;
Diagnosis;
Disease-Free Survival;
Drug Therapy;
Humans;
Induction Chemotherapy;
Medulloblastoma*;
Neoplasm Metastasis;
Neoplasm, Residual;
Neuroectodermal Tumors, Primitive*;
Prognosis;
Retrospective Studies;
Seoul;
Survival Rate*
- From:Korean Journal of Pediatric Hematology-Oncology
2000;7(2):249-255
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
PURPOSE: Children under 3 years old with medulloblastoma (MB) or supratentorial primitive neuroectodermal tumor (SPNET) have a poor prognosis. They are more susceptible to the deleterious effects of craniospinal radiation (CSR). This study was undertaken to determine the survival rates and prognostic factors of currently used treatment. METHPDS: Retrospective review of 19 children (MB 16, SPNET 3) who were newly diagnosed as MB or SPNET at Seoul National University Children's Hospital from April 1996 to August 2000 was done. Eligible Patients were treated with postoperative induction chemotherapy using Children's Cancer Group (CCG) 9921A or 9931. Patients over 3 year of age received CSR as well. RESULTS: Three Patients with SPNET are all alive without disease. However, 6 patients under 3 years old at diagnosis with MB were all dead whereas only one patient had an event among 10 patients over 3 years old at diagnosis. Among patients with MB, age under 3 years at diagnosis or residual tumors after induction chemotherapy correlated with poor outcome. Metastasis at diagnosis, postoperative residual disease or extent of excision did not have a significant effect on survival statistically. Three-year event-free survival (EFS) of patients with MB was 53%. Three-year EFS of patients over 3 years old at diagnosis with MB was 85.7% and 1-year EFS of under 3 years old at diagnosis with MB was 16.7%. CONCLUSION: The combined use of chemotherapy and CSR in the treatment of MB and SPNET improved survival in children over 3 years old, whereas any treatment failed to improve survival in children under 3 years old. So, there's a need for a new treatment modality to improve survival of children under 3 year of age.
