[Medullomyoblastoma: a medulloblastoma with rhabdomyoblastic differentiation].
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.
Gauchotte
Guillaume
G
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
Baylac
François
F
Marie
Béatrice
B
Vignaud
Jean-Michel
JM
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
D014408:Biomarkers, Tumor; D003893:Desmin; D018008:Myogenin; D009363:Neoplasm Proteins; D016900:Neurofilament Proteins; D016708:Synaptophysin