title: WT1 expression in normal and neoplastic cranial and peripheral nerves is independent of grade of malignancy.
abstract: OBJECTIVE
  Wilms' tumor protein (WT1) expression is usually absent in normal glial cells of the CNS but is highly upregulated in brain tumor cells and its expression correlates with tumor grade. However, knowledge on WT1 expression in tumors of the peripheral nerve system (PNS) is limited. As WT1 antibodies not only serve as biomarker for cancerous tissue but also are considered for cancer immunotherapy, knowledge of WT1 expression in tumorous and normal peripheral nerve tissue is important for therapeutical purposes.
  
  
  METHODS
  We analyze the immunohistochemical expression of WT1 in 101 samples consisting of 13 normal nerves, 10 neurofibromas, 69 schwannomas and 9 malignant peripheral nerve sheath tumors (MPNST). Tumor samples included 14 specimen from patients with a proven neurocutaneous disorder (neurofibromatosis type 1 and 2) and 3 cases of schwannomatosis. In 50 vestibular schwannomas tumor growth extension was correlated to WT1 expression.
  
  
  RESULTS
  WT1 expression is present in Schwann cells of the majority of normal human nerves (11/13). In peripheral nerve sheath tumors, cytoplasmic WT1 protein is expressed in the cytoplasm of the neoplastic cells in all tumors, including MPNST, neurofibromas and schwannomas. The WT1 expression is independent of tumor malignancy or tumor growth extension and is not associated with a neurocutaneous disorder.
  
  
  CONCLUSIONS
  WT1 expression in normal and neoplastic tissue differs in the peripheral and the central nervous system. These findings may point to a different functional role of WT1 in the PNS and the CNS.
authors.affiliation: Institute of Brain Research, University of Tuebingen, Tuebingen, Germany. jens.schittenhelm@med.unituebingen.de
authors.forename: Jens
authors.initials: J
authors.lastname: Schittenhelm
authors.forename: John
authors.initials: J
authors.lastname: Thiericke
authors.forename: Christoph
authors.initials: C
authors.lastname: Nagel
authors.forename: Richard
authors.initials: R
authors.lastname: Meyermann
authors.forename: Rudi
authors.initials: R
authors.lastname: Beschorner
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D016022:Case-Control Studies; D002648:Child; D003390:Cranial Nerve Neoplasms / Q000378:metabolism* / Q000473:pathology; D003391:Cranial Nerves / Q000378:metabolism*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D018317:Nerve Sheath Neoplasms / Q000378:metabolism* / Q000473:pathology; D009442:Neurilemmoma / Q000378:metabolism; D009455:Neurofibroma / Q000378:metabolism; D010525:Peripheral Nerves / Q000378:metabolism*; D025721:WT1 Proteins / Q000235:genetics / Q000378:metabolism*; D055815:Young Adult
chemical_list: D025721:WT1 Proteins