High Risk Medulloblastoma in Childhood: Multidisciplinary Approach and Improved Outcome.
- Author:
Joon Sup SONG
1
;
Young Shin RA
;
Seung Do AHN
;
Shin Kwang KHANG
;
Hyun Woo GOO
;
Yoon Jung KIM
;
Mee Jeong LEE
;
Yoon Jeong KIM
;
Thad GHIM
Author Information
1. Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea. tghim@amc.seoul.kr
- Publication Type:Original Article
- Keywords:
Medulloblastoma;
High risk;
Children;
Chemotherapy;
Radiation therapy
- MeSH:
Ataxia;
Brain Neoplasms;
Child;
Chungcheongnam-do;
Cisplatin;
Craniospinal Irradiation;
Cyclophosphamide;
Diagnosis;
Disease-Free Survival;
Drug Therapy;
Etoposide;
Humans;
Hypothyroidism;
Inappropriate ADH Syndrome;
Intellectual Disability;
Intestinal Pseudo-Obstruction;
Medical Records;
Medulloblastoma*;
Mortality;
Neoplasm Metastasis;
Neoplasm, Residual;
Neurosurgery;
Pediatrics;
Puberty, Precocious;
Radiation Oncology;
Retrospective Studies;
Survival Rate;
Vincristine
- From:Korean Journal of Pediatric Hematology-Oncology
2004;11(2):187-194
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
PURPOSE: Medulloblastoma is the most common malignant brain tumor in childhood. The standard treatments are composed of tumor resection, irradiation and chemotherapy. In this study, we analysed the outcome of high risk medulloblastoma patients who were treated with surgical resection followed by craniospinal irradiation and chemotherapy utilizing cisplatin, vincristine, cyclophosphamide and etoposide. METHODS: We conducted a retrospective analysis of medical record of twenty-five patients with high risk medulloblastoma, treated from January 1998 to April 2004 in the Department of Pediatrics, Neurosurgery and Radiation Oncology at Asan Medical Center. RESULTS: The median age at diagnosis was 9 years and 10 month. The 2-year overall survival rate was 80%, and 2-year progression-free survival rate was 71%. Degree of surgical resections or residual tumor did not show statistically significant differences of survival rate, but there was difference depending on metastasis staging. The side effects of chemotherapy were grade IV hematologic toxicity (n=20), SIADH (n=2), and severe paralytic ileus (n=1). The long-term sequelae were endocrinopathy (n=6) that include growth failure, precocious puberty and hypothyroidism. Neurological complications such as mild mental retardation and ataxia occurred in seven patients. There was no treatment-related mortality. Four patients died of tumor progression. CONCLUSION: Patients with high risk medulloblastoma treated with surgical resection followed by radiation and chemotherapy as described here show satisfactory outcome. In this high risk group, metastasis staging correlated with outcome but the degree of surgical resection and presence or absence of residual tumor at primary site did not correlate with outcome.
