title: [Cerebellar gangliocytoma in an 11-year-old child].
abstract: 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 S100 protein. BRAF V600E mutation was absent. Clinical characteristics, radiology, histopathology of the two main diagnoses are discussed.
authors.affiliation: Service d'anatomie et cytologie pathologiques, CHU de Besançon, 3, boulevard Alexandre-Fleming, 25000 Besançon, France.
authors.forename: Marie
authors.initials: M
authors.lastname: Joly
authors.affiliation: Service d'anatomie et cytologie pathologiques, CHU de Besançon, 3, boulevard Alexandre-Fleming, 25000 Besançon, France; Université de Franche-Comté, EA 3181, IFR 133, 19, rue Ambroise-Paré, 25030 Besançon cedex, France. Electronic address: svalmary@univ-fcomte.fr.
authors.forename: Séverine
authors.initials: S
authors.lastname: Valmary-Degano
authors.affiliation: Service de neuroradiologie, CHU de Besançon, 3, boulevard Alexandre-Fleming, 25000 Besançon, France.
authors.forename: Françoise
authors.initials: F
authors.lastname: Cattin
authors.affiliation: Centre de pathologie Est et groupement hospitalier Est, 59, boulevard Pinel, 69677 Bron, France.
authors.forename: Alexandre
authors.initials: A
authors.lastname: Vasiljevic
authors.affiliation: Centre de pathologie Est et groupement hospitalier Est, 59, boulevard Pinel, 69677 Bron, France.
authors.forename: Anne
authors.initials: A
authors.lastname: Jouvet
authors.affiliation: Service d'anatomie et cytologie pathologiques, CHU de Besançon, 3, boulevard Alexandre-Fleming, 25000 Besançon, France; Université de Franche-Comté, EA 3922, 19, rue Ambroise-Paré, 25030 Besançon cedex, France.
authors.forename: Gabriel
authors.initials: G
authors.lastname: Viennet
mesh_terms: D014408:Biomarkers, Tumor / Q000032:analysis; D002528:Cerebellar Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D002648:Child; D003937:Diagnosis, Differential; D020233:Gait Disorders, Neurologic / Q000209:etiology; D005729:Ganglioneuroma / Q000150:complications / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D006223:Hamartoma Syndrome, Multiple / Q000175:diagnosis; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male
chemical_list: D014408:Biomarkers, Tumor
keywords: Cerebellar gangliocytoma; Child; Cowden syndrome; Enfant; Gangliocytome du cervelet; Ganglioglioma; Gangliogliome; Lhermitte-Duclos disease; Maladie de Lhermitte-Duclos; Syndrome de Cowden