Adult anaplastic pilocytic astrocytoma - a diagnostic challenge? A case series and literature review

Fiechter, Michael; Hewer, Ekkehard; Knecht, Urspeter; Wiest, Roland; Beck, Jürgen; Raabe, Andreas; Oertel, Markus Florian (2016). Adult anaplastic pilocytic astrocytoma - a diagnostic challenge? A case series and literature review. Clinical neurology and neurosurgery, 147, pp. 98-104. Elsevier 10.1016/j.clineuro.2016.06.005

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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.


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.


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.


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.

Item Type:

Journal Article (Review Article)


04 Faculty of Medicine > Department of Head Organs and Neurology (DKNS) > Clinic of Neurosurgery
04 Faculty of Medicine > Department of Radiology, Neuroradiology and Nuclear Medicine (DRNN) > Institute of Diagnostic and Interventional Neuroradiology
04 Faculty of Medicine > Service Sector > Institute of Pathology > Clinical Pathology

UniBE Contributor:

Fiechter, Michael, Hewer, Ekkehard Walter, Knecht, Urspeter, Wiest, Roland Gerhard Rudi, Beck, Jürgen, Raabe, Andreas, Oertel, Markus Florian


600 Technology > 610 Medicine & health








Martin Zbinden

Date Deposited:

13 Sep 2016 14:29

Last Modified:

02 Mar 2023 23:28

Publisher DOI:


PubMed ID:


Uncontrolled Keywords:

Adult; Anaplastic; Diagnosis; Glioma; Pilocytic astrocytoma; Rare




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