title: Supratentorial primitive neuroectodermal tumours of the brain: multidirectional differentiation does not influence prognosis. A clinicopathological report of 18 patients.
abstract: OBJECTIVE
  To examine the clinical and pathological characteristics of supratentorial primitive neuroectodermal tumours (PNETs) in a retrospective series of 18 patients, according to the strict definition of the World Health Organization classification of tumours that excludes other types of malignant embryonal tumours of the brain.
  
  
  RESULTS
  Eleven children and seven adults with supratentorial PNETs were diagnosed between 1993 and 2002 and their medical records were reviewed. An immunohistochemical study was performed on formalin-fixed paraffin-embedded tissue of 18 primary tumours and five recurrences with antibodies for neuronal (neuron specific enolase, synaptophysin, neurofilament, chromogranin A), epithelial [epithelial membrane antigen (EMA), cytokeratin], glial [glial fibrillary acidic protein (GFAP)], muscle (desmin, h-caldesmon, alpha-smooth muscle actin, myogenin) differentiation and with two anti-CD99 antibodies. All tumours showed at least one neuronal marker except chromogranin A; a variable number of cells were GFAP+ or EMA+ in 18/23 tumours. Six primary tumours and one recurrence were positive for cytokeratin and/or one muscle antigen except myogenin. CD99 was observed in 33% of the cases. The mean duration of overall survival was 20 months. The estimated overall survival rates were 61% at 1 year, 29% at 2 years, and 18% at 3 years. Two factors of poor prognosis were identified by univariate analysis: a positive cerebrospinal fluid cytology at diagnosis and the absence of complete resection. No distinct immunophenotype was statistically related to survival.
  
  
  CONCLUSIONS
  A multidirectional differentiation is a frequent event in supratentorial PNETs but has no apparent influence on the outcome of this aggressive neoplasm.
authors.affiliation: Department of Pathology, Centre of Clinical Research, University Hospital of Angers, Angers, France.
authors.forename: S
authors.initials: S
authors.lastname: Visée
authors.forename: C
authors.initials: C
authors.lastname: Soltner
authors.forename: X
authors.initials: X
authors.lastname: Rialland
authors.forename: M C
authors.initials: MC
authors.lastname: Machet
authors.forename: D
authors.initials: D
authors.lastname: Loussouarn
authors.forename: S
authors.initials: S
authors.lastname: Milinkevitch
authors.forename: A
authors.initials: A
authors.lastname: Pasco-Papon
authors.forename: P
authors.initials: P
authors.lastname: Mercier
authors.forename: M C
authors.initials: MC
authors.lastname: Rousselet
mesh_terms: D000071443:12E7 Antigen; D000293:Adolescent; D000328:Adult; D015703:Antigens, CD / Q000032:analysis; D001932:Brain Neoplasms / Q000378:metabolism / Q000473:pathology*; D015815:Cell Adhesion Molecules / Q000032:analysis; D002454:Cell Differentiation; D002648:Child; D002675:Child, Preschool; D005260:Female; D005904:Glial Fibrillary Acidic Protein / Q000032:analysis; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D008297:Male; D008875:Middle Aged; D018396:Mucin-1 / Q000032:analysis; D018242:Neuroectodermal Tumors, Primitive / Q000378:metabolism / Q000473:pathology*; D016900:Neurofilament Proteins / Q000032:analysis; D010751:Phosphopyruvate Hydratase / Q000032:analysis; D011379:Prognosis; D016019:Survival Analysis; D016708:Synaptophysin / Q000032:analysis
chemical_list: D000071443:12E7 Antigen; D015703:Antigens, CD; C061828:CD99 protein, human; D015815:Cell Adhesion Molecules; D005904:Glial Fibrillary Acidic Protein; D018396:Mucin-1; D016900:Neurofilament Proteins; D016708:Synaptophysin; D010751:Phosphopyruvate Hydratase