Twelve Clinically Significant Points in Medulloblastoma(<SPECIAL ISSUE>Pediatric Neurosurgery and Long-term Prognosis)

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  • Sugiyama Kazuhiko
    Department of Neurosurgery, Graduate School of Biomedical Sciences Hiroshima University
  • Yamasaki Fumiyuki
    Department of Neurosurgery, Graduate School of Biomedical Sciences Hiroshima University
  • Kajiwara Yoshinori
    Department of Neurosurgery, Graduate School of Biomedical Sciences Hiroshima University
  • Watanabe Yosuke
    Department of Neurosurgery, Graduate School of Biomedical Sciences Hiroshima University
  • Takayasu Takeshi
    Department of Neurosurgery, Graduate School of Biomedical Sciences Hiroshima University
  • Kurisu Kaoru
    Department of Neurosurgery, Graduate School of Biomedical Sciences Hiroshima University

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  • 髄芽腫臨床を理解するための12項目(<特集>小児神経外科治療と長期予後)

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Though medulloblastoma is the most common malignant brain tumor during childhood, only 80 newly-diagnosed tumors develos every year, as the annual incidence is extremely rare with an occurrence of 0.5 per 100,000 children younger than 15-year-old and of 0.7 per 100,000 for the entire population. Images obtained of medulloblastoma are characterized by a round heterogeously-enhanced mass in or adjacent to the VIth ventricle. Objectives of surgical treatment are the maximum resectioning of the main mass and the relief of the obstructive hydrocephalus. Cerebellar mutism occurs a few days after one fourth of medulloblastoma surgery, and lasts approximately for 50 days followed by subsequent dysarthria. Pathological subtypes include classic medulloblastoma, desmoplastic/nodular medulloblastoma, medulloblastoma with extensive nodularity, large cell/anaplastic medulloblastoma, all corresponding to WHO grade IV. According to age, residual tumor size, and disseminated staging, patients are divided into average-risk group, high-risk group, or baby-medulloblastoma after surgery. Standard treatment in average-risk group includes 23.4-Gy cranio-spinal irradiation (CSI) with posterior boost followed by chemotherapy consisting of CDDP, alkylating agents, and vincristine. Patients in high-risk group receive over 36-Gy CSI with boost radiotherapy to nodular lesions before, concomitantly with, or followed by dose-intensity chemotherapy. In cases with gross total removal, or desmoplastic/nodular pathology, radiotherapy for patients younger than 3-year-old are often delayed until they turn 3-year-old, and are able to survive for long time by appropriate chemotherapy alone. Adolescent survivors with childhood medulloblastoma have a number of late adverse effects regarding another neoplasm, neuro-cognitive function, endocrine activity, cardiovascular organs, and skeletal system. Comprehensive follow-up and support system are mandatory.

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