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@ARTICLE{Abe:277309,
author = {M. Abe and T. Ono and F. Hinz$^*$ and M. Takahashi and Y.
Hiroshima and K. Kodama and M. Yano and H. Nanjo and T.
Takahashi and A. von Deimling$^*$ and H. Shimizu},
title = {{D}etailed molecular and pathological analyses of primary
intracranial embryonal rhabdomyosarcoma with a {BRAF}
mutation: illustrative case.},
journal = {Journal of neurosurgery / Case lessons},
volume = {6},
number = {1},
issn = {2694-1902},
address = {Charlottesville, Va.},
publisher = {American Association of Neurological Surgeons},
reportid = {DKFZ-2023-01327},
pages = {CASE23207},
year = {2023},
note = {2023 Jul 3;6(1):CASE23207},
abstract = {The etiological significance of the RAS and PI3K pathways
has been reported in systemic embryonal rhabdomyosarcoma
(ERMS) but not in primary intracranial ERMS (PIERMS).
Herein, the authors present a unique case of PIERMS with a
BRAF mutation.A 12-year-old girl with progressive headache
and nausea was diagnosed with a tumor in the right parietal
lobe. Semi-emergency surgery revealed an intra-axial lesion
that was histopathologically identical to an ERMS.
Next-generation sequencing indicated a BRAF mutation as a
pathogenic variation, but the RAS and PI3K pathways showed
no alteration. Although there is no established reference
class for PIERMS, the DNA methylation prediction was closest
to that of ERMS, indicating the possibility of PIERMS. The
final diagnosis was PIERMS. The patient underwent local
radiotherapy (50.4 Gy) and multiagent chemotherapy, with no
recurrence for 12 months after surgery.This may be the first
case demonstrating the molecular features of PIERMS,
especially the intra-axial type. The results showed a
mutation in BRAF but not in the RAS and PI3K pathways, which
is different from the existing ERMS features. This molecular
difference may cause differences in DNA methylation
profiles. Accumulation of the molecular features of PIERMS
is necessary before any conclusions can be drawn.},
keywords = {BRAF (Other) / intra-axial tumor (Other) / methylation
profile (Other) / next-generation sequencing (Other) /
rhabdomyosarcoma (Other)},
cin = {B300 / HD01},
ddc = {610},
cid = {I:(DE-He78)B300-20160331 / I:(DE-He78)HD01-20160331},
pnm = {312 - Funktionelle und strukturelle Genomforschung
(POF4-312)},
pid = {G:(DE-HGF)POF4-312},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:37392767},
doi = {10.3171/CASE23207},
url = {https://inrepo02.dkfz.de/record/277309},
}