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Pediatric Blood and Cancer 2008-Nov

Characteristics and outcomes of medulloblastoma in adults.

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Celina Ang
David Hauerstock
Marie-Christine Guiot
Goulnar Kasymjanova
David Roberge
Petr Kavan
Thierry Muanza

Słowa kluczowe

Abstrakcyjny

BACKGROUND

Adult medulloblastoma is a rare disease for which there is no internationally accepted standard of care. Treatment regimens have typically been modeled after pediatric protocols. We sought to review the presentation, management, and outcome of patients with adult medulloblastoma treated at the McGill University teaching hospitals over the past 18 years.

METHODS

Medical records were reviewed to gather demographic and clinical data including presenting symptoms, tumor characteristics, management, survival, and treatment toxicity.

RESULTS

Twenty-five patients were identified. Eleven patients were female and 14 were male. The median age at diagnosis was 30 (range 17-48). Our 5- and 10-year overall survival (OS) rates were 78% and 30%, respectively. Median OS was 108 months and median progression-free survival time was 63 months. Age, sex, risk, stage, extent of resection, chemotherapy and time between surgery and adjuvant therapy did not significantly influence survival outcomes. The most frequently reported adverse events included sensory neuropathy, nausea, vomiting, febrile neutropenia, and radiation dermatitis.

CONCLUSIONS

Adult medulloblastoma has distinct characteristics from the pediatric population including presentation in the lateral cerebellar hemispheres. Late relapses, especially in the posterior fossa, are a significant problem. Further follow-up will be required to ascertain the effect of adjuvant chemotherapy on survival.

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