title: [Medullomyoblastoma: a medulloblastoma with rhabdomyoblastic differentiation].
abstract: A 26 years old patient was operated for a tumor of cerebellar vermix, and then reoperated for a relapse at the age of 35 years, with a similar histological pattern in both cases. At pathologic examination, the tumor was composed of hypercellular sheets typical of medulloblastoma, containing also sparse large cells with eosinophilic cytoplasm and round nuclei containing voluminous nucleoli. Neuroblastic cells showed expression of neurofilament protein and synaptophysin. The large cells expressed desmin, myogenin, and neurofilament. These morphological and immunohistochemical features are characteristic of medullomyoblastoma. The patient deceased 11 years after the initial surgery. Medullomyoblastoma is a rare variant of medulloblastoma with a rhabdomyoblastic differentiation. The two tumoral populations share the same genetic alterations. The main differential diagnoses are atypical teratoid/rhabdoid tumor, immature teratoma, medulloepithelioma, primitive intracranial rhabdomyosarcoma and myoneurocytoma.
authors.affiliation: Laboratoire d'anatomie et cytologie pathologiques, hôpital Central, CHU de Nancy, 29, avenue du Maréchal-de-Lattre-de-Tassigny, 54000 Nancy, France. g.gauchotte@yahoo.fr
authors.forename: Guillaume
authors.initials: G
authors.lastname: Gauchotte
authors.forename: François
authors.initials: F
authors.lastname: Baylac
authors.forename: Béatrice
authors.initials: B
authors.lastname: Marie
authors.forename: Jean-Michel
authors.initials: JM
authors.lastname: Vignaud
mesh_terms: D000328:Adult; D014408:Biomarkers, Tumor / Q000032:analysis; D002454:Cell Differentiation; D002528:Cerebellar Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D003893:Desmin / Q000032:analysis; D018450:Disease Progression; D017809:Fatal Outcome; D006801:Humans; D008297:Male; D008527:Medulloblastoma / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D032342:Muscle Cells / Q000737:chemistry / Q000473:pathology; D018008:Myogenin / Q000032:analysis; D009363:Neoplasm Proteins / Q000032:analysis; D009364:Neoplasm Recurrence, Local / Q000737:chemistry / Q000473:pathology / Q000601:surgery; D016900:Neurofilament Proteins / Q000032:analysis; D012208:Rhabdomyosarcoma / Q000473:pathology; D016708:Synaptophysin / Q000032:analysis
chemical_list: D014408:Biomarkers, Tumor; D003893:Desmin; D018008:Myogenin; D009363:Neoplasm Proteins; D016900:Neurofilament Proteins; D016708:Synaptophysin