Jong Hyung Yoon1,2, Kyung Duk Park1, Hyoung Jin Kang1, Hyery Kim1, Ji Won Lee1, Seung-Ki Kim3, Kyu-Chang Wang3, Sung-Hye Park4, Il Han Kim5, Hee Young Shin6,7. 1. Department of Pediatrics, Cancer Research Institute, New York, USA. 2. Department of Pediatrics, Hallym University College of medicine, Chuncheon Sacred Heart Hospital, Chuncheon, Korea. 3. Department of Neurosurgery, Seoul National University College of Medicine, Seoul, Korea. 4. Department of Pathology, Seoul National University College of Medicine, Seoul, Korea. 5. Department of Radiation Oncology, Seoul National University College of Medicine, Seoul, Korea. 6. Department of Pediatrics, Cancer Research Institute, New York, USA. hyshin@snu.ac.kr. 7. Division of Hematology/Oncology, Department of Pediatrics, Seoul National University College of Medicine, Seoul National University Children's Hospital, 101 Daehak-ro, Jongno-gu, Seoul, 03080, Korea. hyshin@snu.ac.kr.
Abstract
BACKGROUND: Although craniospinal irradiation (CSI) of 2340 cGy plus tumor booster with chemotherapy have been established as a standard treatment of childhood average-risk (AvR) medulloblastoma (MBL) in Western counties, there are a few recent reports in outcomes of AvR MBL using this strategy in Korean and other Asian children. We investigated the outcome of the Korean children with AvR MBL who were treated with CSI <2500 cGy and chemotherapy. METHODS: Between January 2001 and December 2010, clinical characteristics and outcomes of 42 patients who were diagnosed with AvR MBL postoperatively and treated with radiation including CSI <2500 cGy and chemotherapy in Seoul National University Children's Hospital were analyzed. RESULTS: Their median age was 9 years (range: 3-18.8), and 29 were male. Histological subtypes were classic type in 28 patients, nodular/desmoplastic in 7, and large cell/anaplastic (LCA) in 7. All the patients received adjuvant radiotherapy (CSI with median 2340 cGy and booster) and multiagent chemotherapy as the first-line treatment. With a median follow-up of 54 months, 12 patients experienced relapse or progression of the tumor. The 3- and 5-year disease-free survival (DFS) rates were 78.0%±6.5% and 75.0%±6.9%, respectively, and overall survival (OS) rates were 85.3%±5.6% and 76.8%±6.9%, respectively. The LCA subtype was associated with poorer DFS (P=0.023) and OS (P=0.008), compared with non-LCA subtypes. CONCLUSIONS: The outcomes of children and adolescents with AvR MBL treated with radiation including CSI <2500 cGy and chemotherapy, are compatible to those in Western countries; however, the LCA subtype has a poor outcome with this strategy.
BACKGROUND: Although craniospinal irradiation (CSI) of 2340 cGy plus tumor booster with chemotherapy have been established as a standard treatment of childhood average-risk (AvR) medulloblastoma (MBL) in Western counties, there are a few recent reports in outcomes of AvR MBL using this strategy in Korean and other Asian children. We investigated the outcome of the Korean children with AvR MBL who were treated with CSI <2500 cGy and chemotherapy. METHODS: Between January 2001 and December 2010, clinical characteristics and outcomes of 42 patients who were diagnosed with AvR MBL postoperatively and treated with radiation including CSI <2500 cGy and chemotherapy in Seoul National University Children's Hospital were analyzed. RESULTS: Their median age was 9 years (range: 3-18.8), and 29 were male. Histological subtypes were classic type in 28 patients, nodular/desmoplastic in 7, and large cell/anaplastic (LCA) in 7. All the patients received adjuvant radiotherapy (CSI with median 2340 cGy and booster) and multiagent chemotherapy as the first-line treatment. With a median follow-up of 54 months, 12 patients experienced relapse or progression of the tumor. The 3- and 5-year disease-free survival (DFS) rates were 78.0%±6.5% and 75.0%±6.9%, respectively, and overall survival (OS) rates were 85.3%±5.6% and 76.8%±6.9%, respectively. The LCA subtype was associated with poorer DFS (P=0.023) and OS (P=0.008), compared with non-LCA subtypes. CONCLUSIONS: The outcomes of children and adolescents with AvR MBL treated with radiation including CSI <2500 cGy and chemotherapy, are compatible to those in Western countries; however, the LCA subtype has a poor outcome with this strategy.
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