title: Medulloblastomas-primitive neuroectodermal tumours in the adult population.
abstract: Medulloblastomas - primitive neuroectodermal tumours are rare in adults. This review will evaluate a single centre's experience with this malignancy. The medulloblastoma - primitive neuroectodermal tumour database was evaluated for all patients aged more than 18 years who were referred for management. Relevant information from the database was abstracted to provide a descriptive record of this malignancy. Between 1977-2004 there were 11 patients referred, 1 with persistent disease and 10 were eligible, presenting with initial diagnosis. There was increased intracranial pressure in 50% of patients. Most patients had symptoms >3 months, with three having symptoms 1-3 months before diagnosis and one patient having thoracic dural metastases at presentation. Complete resection was recorded in four patients and six had 50-90% resection. All patients completed craniospinal radiotherapy (35-36 Gy at 1.8-2.0 Gy fractions) barring one patient, who died of surgical complications during his radiotherapy. Chemotherapy was given in five of the nine patients postradiotherapy. There were two posterior fossa recurrences, with associated supratentorial and extra central nervous system disease. Of the 10 primary patients 7 are alive with no evidence of disease, 2 died because of disease, with 1 intercurrent death. One patient developed a second malignancy. The outcome for adults matches that of the more common paediatric patients. Radiotherapy could control local disease even where complete resection was not achieved.
authors: Department of Radiation Oncology, Prince of Wales Hospital, Sydney, NSW, Australia. robert.smee@sesiahs.health.nsw.gov.au
authors: R I
authors: RI
authors: Smee
authors: J R
authors: JR
authors: Williams
mesh_terms: D000293:Adolescent; D000328:Adult; D002528:Cerebellar Neoplasms / Q000453:epidemiology* / Q000628:therapy; D016208:Databases, Factual / Q000706:statistics & numerical data; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007427:Intracranial Pressure; D008297:Male; D008527:Medulloblastoma / Q000453:epidemiology* / Q000628:therapy; D009364:Neoplasm Recurrence, Local; D018242:Neuroectodermal Tumors, Primitive / Q000453:epidemiology* / Q000628:therapy; D019635:Neurosurgical Procedures; D009517:New South Wales / Q000453:epidemiology; D018714:Radiotherapy, Adjuvant; D035583:Rare Diseases; D012189:Retrospective Studies; D016896:Treatment Outcome