Abstract

Medullomyoblastoma is an extremely rare tumor, corresponding to a variant of medulloblastoma with rhabdomyoblastic differentiation. Both tumor populations are sharing the same genetic alterations. We report a case of a 10 year-old girl, admitted for symptoms of intracranial hypertension associated with a cerebellar syndrome. Magnet resonance imaging of the brain was performed, and revealed a lesion of the cerebellar vermis. A wide excision was performed and the histopathological examination objectified a densely round cells proliferation coexisting with nests of rhabdomyoblastic cells that express desmin and myogenin in immunohistochemistry, making the diagnosis of medullomyoblastoma.

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