Cerebellar gangliocytoma in an 11-year-old child

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TitreCerebellar gangliocytoma in an 11-year-old child
Type de publicationJournal Article
Year of Publication2014
AuteursJoly M, Valmary-Degano S, Cattin F, Vasiljevic A, Jouvet A, Viennet G
JournalANNALES DE PATHOLOGIE
Volume34
Pagination477-480
Date PublishedDEC
Type of ArticleArticle
ISSN0242-6498
Mots-clésCerebellar gangliocytoma, Child, Cowden syndrome, Ganglioglioma, Lhermitte-duclos disease
Résumé

Cerebellar gangliocytoma can correspond to Lhermitte-Duclos disease, a benign hamartomatous malformation encountered in young adults. It can also be a part of gangliogliomas/gangliocytomas family, which usually encompasses temporal pediatric neoplasms associated with longstanding seizures. We report a case of a young 11-year-old patient who presented with a gangliocytoma of the cerebellum revealed by neurologic manifestations (headache, dyspraxia, equilibrium and gait disturbances). Diagnosis was made on surgical material. Tumour was characterized by dysplastic mature ganglion cells, perivascular lymphocytic infiltrates and no glial neoplastic component. By immunohistochemistry, ganglion cells expressed neurofilaments, MAP2 protein, synaptophysin, chromogranin A and 5100 protein. BRAF V600E mutation was absent. Clinical characteristics, radiology, histopathology of the two main diagnoses are discussed. (C) 2014 Elsevier Masson SAS. All rights reserved.

DOI10.1016/j.annpat.2014.09.008