title: Adult anaplastic pilocytic astrocytoma - a diagnostic challenge? A case series and literature review.
abstract: BACKGROUND
  Anaplastic pilocytic astrocytoma (APA) is an exceptionally rare type of high-grade glioma in adults. Establishing histopathological diagnosis is challenging and its clinical and radiological appearance insidious. By this case series and first literature review we investigated the various clinical, neuroradiological, and histopathological features of APA in adults.
  
  
  METHODS
  An in hospital screening of the database from the Institute of Pathology was conducted to identify cases of APA. Further, we performed a literature review in PubMed using the keywords "anaplastic/malignant/atypical AND pilocytic astrocytoma" and "anaplastic astrocytoma/glioblastoma AND Rosenthal fibers" and summarized the current knowledge about APA in adults.
  
  
  RESULTS
  Over the last decade we were able to identify 3 adult patients with APA in our hospital. According to the pertinent literature, the prognosis of APA in adults (documented survival of up to 10 years) appears to be better than in other high-grade gliomas. Few cases were associated with neurofibromatosis type 1, which seems to predispose for development of APA. Although molecular genetics is still of limited value for differentiation of APA from other high-grade glioma, advanced neuroimaging techniques such as magnetic resonance perfusion imaging and spectroscopy allow improved differential work-up. In particular, APA in adults has the ability to mimic various neurological diseases such as tumefactive demyelinating lesions, low-, or high-grade gliomas.
  
  
  CONCLUSIONS
  Although currently not explicitly recognized as a distinct clinico-pathologic entity it seems that adult APA behaves differently from conventional high-grade glioma and should be included in differential diagnostics to enable adequate patient care. However, further studies are needed to better understand this extremely rare disease.
authors.affiliation: Department of Neurosurgery, Inselspital, Bern University Hospital and University of Bern, Bern, Switzerland.
authors.forename: Michael
authors.initials: M
authors.lastname: Fiechter
authors.affiliation: Institute of Pathology, University of Bern, Bern, Switzerland.
authors.forename: Ekkehard
authors.initials: E
authors.lastname: Hewer
authors.affiliation: Institute for Diagnostic and Interventional Neuroradiology, Inselspital, Bern University Hospital and University of Bern, Bern, Switzerland.
authors.forename: Urspeter
authors.initials: U
authors.lastname: Knecht
authors.affiliation: Institute for Diagnostic and Interventional Neuroradiology, Inselspital, Bern University Hospital and University of Bern, Bern, Switzerland.
authors.forename: Roland
authors.initials: R
authors.lastname: Wiest
authors.affiliation: Department of Neurosurgery, Inselspital, Bern University Hospital and University of Bern, Bern, Switzerland.
authors.forename: Jürgen
authors.initials: J
authors.lastname: Beck
authors.affiliation: Department of Neurosurgery, Inselspital, Bern University Hospital and University of Bern, Bern, Switzerland.
authors.forename: Andreas
authors.initials: A
authors.lastname: Raabe
authors.affiliation: Department of Neurosurgery, Inselspital, Bern University Hospital and University of Bern, Bern, Switzerland. Electronic address: markus.oertel@insel.ch.
authors.forename: Markus F
authors.initials: MF
authors.lastname: Oertel
mesh_terms: D000328:Adult; D001254:Astrocytoma / Q000175:diagnosis*; D001932:Brain Neoplasms / Q000175:diagnosis*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged
keywords: Adult; Anaplastic; Diagnosis; Glioma; Pilocytic astrocytoma; Rare