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@ARTICLE{Sahm:143136,
author = {F. Sahm$^*$ and D. E. Reuss$^*$ and C. Giannini},
title = {{WHO} 2016 classification: changes and advancements in the
diagnosis of miscellaneous primary {CNS} tumours.},
journal = {Neuropathology $\&$ applied neurobiology},
volume = {44},
number = {2},
issn = {0305-1846},
address = {Oxford [u.a.]},
publisher = {Wiley-Blackwell},
reportid = {DKFZ-2019-00745},
pages = {163 - 171},
year = {2018},
abstract = {This short review highlights significant changes and recent
findings incorporated to varying extent in the WHO 2016
definition of a variety of tumours, including peripheral
nerve sheath tumours, meningiomas, mesenchymal
nonmeningothelial tumours, melanocytic tumours, lymphomas
and histiocytic tumours, germ cell tumours and
non-neuroendocrine pituitary tumours. Most notable
classification changes include: adding hybrid nerve sheath
tumours to the spectrum of benign nerve sheath tumours; an
updated definition of atypical meningioma (WHO grade II),
including cases with brain invasion; recognizing dural
solitary fibrous tumour (SFT) and haemangiopericytoma (HPC)
as a single tumour entity characterized by NAB2 and STAT6
gene fusions for which the term SFT/HPC was chosen;
recognizing that pituitary granular cell tumour, spindle
cell oncocytoma, and pituicytoma all share nuclear
expression of TTF-1, possibly representing a spectrum of a
single nosological entity derived from posterior pituitary
glial cells. The most significant diagnostic markers which
have emerged include: inactivation of NF1, CDKN2A, and PRC2
components, SUZ12 and EED in MPNST, leading to neurofibromin
and H3K27me3 expression loss; GNAQ and GNA11 mutations in
CNS primary melanocytic tumours; BRAFV600E mutation in
histiocytic tumours (Langerhans cell histiocytosis and
Erdheim-Chester disease) and papillary craniopharyngioma,
which provides both a diagnostic marker in the appropriate
pathological setting and a therapeutic target. The WHO 2016
Classification has balanced cutting-edge knowledge on the
molecular characteristics of the miscellaneous CNS tumours
reviewed here with a practical approach for their daily
diagnostic work-up. Much more progress can be expected in
the classification of these neoplasms in the near future.},
subtyp = {Review Article},
cin = {G380},
ddc = {610},
cid = {I:(DE-He78)G380-20160331},
pnm = {317 - Translational cancer research (POF3-317)},
pid = {G:(DE-HGF)POF3-317},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:28295484},
doi = {10.1111/nan.12397},
url = {https://inrepo02.dkfz.de/record/143136},
}