B Grassiot, P A Beuriat, F Di Rocco, P Leblond, C Faure-Conter, A Szathmari, C Mottolese
Neuro-Chirurgie 2021 January 19

INTRODUCTION: Modern approach for the treatment of posterior fossa medulloblastomas remains a challenge for pediatric neurosurgeons and pediatric oncologists and requires a multidisciplinary approach to optimize survival and clinical results.

MATERIAL AND METHODS: We report the surgical principles of the treatment of posterior fossa medulloblastomas in children and how to avoid technical mistakes especially in very young patients. We also report our experience in a series of 64 patients operated from a medulloblastoma between 2000 and 2018 in Lyon.

RESULTS: All patients had a cranio-spinal MRI. Eighty-one percent of the patients (n=50) had strictly midline tumor while 19% (n=14) had lateralized one. Eleven percent (n=7) had metastasis at diagnosis on the initial MRI. Forty-one percent (n=29) had an emergency ETV to treat hydrocephaly and the intracranial hypertension. All patient underwent a direct approach and a complete removal was achieved in 78% (n=58) of the cases on the post-operative MRI realized within 48h post-surgery. Histological findings revealed classical medulloblastoma in 73% (n=46), desmoplastic medulloblastoma in 17% (n=11) and anaplastic/large cell medulloblastoma in 10% (n=7). Patient were classified as low risk in 7 cases, standard risk in 30 cases and high risk in 27 cases. Ninety-six percent (n=61) of the patient received radiotherapy. Seventy-six percent (n=48) received pre-irradiation or adjuvant chemotherapy. At last follow-up in December 2018, 65% (n=41) of the patient were in complete remission, 12% (n=8) were in relapse and 27% (n=15) had died from their disease. The overall survival at five et ten and fifteen years for all the series was of 76 %, 73% and 65,7%.

CONCLUSIONS: Medulloblastomas remain a chimio-sensible and radio-sensible disease and the complete surgical removal represents a favorable prognostic factor. The extension of surgery has also to be weighted in consideration of the new bio-molecular and genetic knowledge that have to be integrated by surgeons to improve quality of life of patients.

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