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@ARTICLE{Appay:156763,
author = {R. Appay and M. Pages and C. Colin and D. T. W. Jones$^*$
and P. Varlet and D. Figarella-Branger},
title = {{D}iffuse leptomeningeal glioneuronal tumor: a double
misnomer? {A} report of two cases.},
journal = {Acta Neuropathologica Communications},
volume = {8},
number = {1},
issn = {2051-5960},
address = {London},
publisher = {Biomed Central},
reportid = {DKFZ-2020-01087},
pages = {95},
year = {2020},
abstract = {Diffuse leptomeningeal glioneuronal tumor (DLGNT) was
introduced, for the first time, as a provisional entity in
the 2016 WHO classification of central nervous system
tumors. DLGNT mainly occur in children and characterized by
a widespread leptomeningeal growth occasionally associated
with intraspinal tumor nodules, an oligodendroglial-like
cytology, glioneuronal differentiation and MAP-Kinase
activation associated with either solitary 1p deletion or
1p/19q codeletion in the absence of IDH mutation.We report
here two unexpected DLGNTs adult cases, characterized by a
unique supratentorial circumscribed intraparenchymal tumor
without leptomeningeal involvement in spite of long
follow-up. In both cases, the diagnosis of DLGNT was made
after DNA-methylation profiling which demonstrated that one
case belonged to the DLGNT class whereas the other remained
not classifiable but showed on CNV the characteristic
genetic findings recorded in DLGNT. Both cases harbored
1p/19q codeletion associated with KIAA1549:BRAF fusion in
one case and with BRAF V600E and PIK3CA E545A mutations, in
the other.Our study enlarges the clinical and molecular
spectrum of DLGNTs, and points out that the terminology of
DLGNTs is not fully appropriate since some cases could have
neither diffuse growth nor leptomeningeal dissemination.
This suggests that DLGNTs encompass a wide spectrum of
tumors that has yet to be fully clarified.},
cin = {B360},
ddc = {610},
cid = {I:(DE-He78)B360-20160331},
pnm = {312 - Functional and structural genomics (POF3-312)},
pid = {G:(DE-HGF)POF3-312},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:32605662},
doi = {10.1186/s40478-020-00978-7},
url = {https://inrepo02.dkfz.de/record/156763},
}