Medulloblastoma is the most common childhood intracranial tumor, accounting for 20 to 25% of pediatric intracranial tumors reported in the United Kingdom.55 During the past 30 years, radical surgical removal of the tumor followed by radiotherapy to the craniospinal axis, with or without complementary chemotherapy, has been responsible for an improvement in the 5-year survival rate to 50% or better (Tables 1 and 2).4,6,7,15,16,18,21,26–28,35,43,47,52
|Authors & Year*||No. of Cases||5-Year Survival (%)||Periop Mortality (%)||Result of Spinal Investigation†|
|Hirsch, et al., 1979||57||54||10.5||not given|
|cohort 1 (no XRT)||46||2||not given||not given|
|cohort 2 (XRT)||41||39||not given||not given|
|Raimondi & Tomita, 1979||51||34.5||8||not given|
|Choux, et al., 1982||60||37||not given||not given|
|Hoffman, et al., 1982||144||47||not given||not given|
|Hoppe-Hirsch, et al., 1990||120||60||6.5||3% (3 of 105)|
|Jenkin, et al., 1990||72||64||not given||13% (2 of 15)|
|Di Rocco, et al., 1994||45||44.4||not given||not given|
|Cervoni & Cantore, 1995||35||60||14||not given|
|Sure, et al., 1995||66||50||not given||14% (9 of 66)|
|David, et al., 1997||80||50||5||33% (25 of 76)|
|metaanalysis||817||45.2||8.8||15% (39 of 262)|
XRT = radiotherapy.
The proportion of patients with positive myelography (the absolute number of positive myelography/all myelography; range 3–33%) where it was declared. In 67.9% (555 cases) the presence or absence of metastases was not declared.
|Authors & Year||No. of Cases||5-Year Survival (%)||Adjuvant Therapy||Indications†||Results of Spinal Investigation‡|
|Bloom, et al., 1969||82||32||XRT||all patients||not given|
|Harisiadis & Chang, 1977||58||40.4||XRT||all patients||26% (15 of 58)|
|Hughes, et al., 1988||60||68||XRT||partial removal, brainstem involvement, <2 yrs of age||not given|
|Evans, et al. (CCSG &||233||59||XRT & CHT||metastases, advanced local disease, <4 yrs of age||18% (42 of|
|Tait, et al. (SIOP), 1990||286||53||XRT & CHT||partial removal, brainstem involvement, Chang stages T3 & T4||not given|
|Halberg, et al., 1991||65||73||low-dose XRT & CHT||>75% resection, no metastases, >2 yrs of age||not given|
|Krischer, et al., 1991||71||68||XRT & CHT||>5 yrs of age, males||not given|
|Duffner, et al., 1993||62||46 (at||CHT & XRT||CHT <3 yrs of age, to delay XRT||not given|
|Packer, et al., 1994||63||83||XRT & CHT||all patients||17% (11 of 63)|
|Wara, et al., 1994||92||52||XRT & CHT||all patients||not given|
|Gentet, et al., 1995||68||62||XRT & CHT||partial removal, brainstem involvement, <2 yrs of age||24% (16 of 68)|
CCSG = Children's Cancer Study Group; CHT = chemotherapy; NA = not applicable; RTOG = Radiation Therapy Oncology Group; SIOP = International Society of Pediatric Oncology; XRT = radiotherapy.
Recommendations for use of protocol concluded by study.
Proportion of patients with positive myelography (the absolute number of positive myelography/all myelography; range 17–26%) where it was declared.
In 1979 McIntosh35 reported 87 consecutive cases of medulloblastoma operated on at Great Ormond Street Hospital for Children (GOSH) between 1965 and 1974. The continuing large number of cases seen here allows us a unique opportunity to view longitudinally the effect that modern methods of investigation and therapy have had on the prognosis of this disease. We report here the details of 80 cases diagnosed, operated on, and followed in the 10 years from 1980 to 1990 at this hospital in an attempt to determine whether any improvement has occurred in the observed disease—free survival rate for medulloblastoma since McIntosh's earlier report.
Rorke LB: The cerebellar medulloblastoma and its relationship to primitive neuroectodermal tumors. J Neuropathol Exp Neurol 42:1–151983Rorke LB: The cerebellar medulloblastoma and its relationship to primitive neuroectodermal tumors. J Neuropathol Exp Neurol 42:
Dr. David's work was supported by the Department of Neurosurgery, University of Péecs Medical School, Hungary, while he served as a registrar at GOSH.