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@ARTICLE{Hirsch:172546,
author = {S. Hirsch and N. Dikow and S. M. Pfister$^*$ and K.
Pajtler$^*$},
title = {{C}ancer predisposition in pediatric
neuro-oncology-practical approaches and ethical
considerations.},
journal = {Neuro-oncology practice},
volume = {8},
number = {5},
issn = {2054-2585},
address = {Oxford},
publisher = {Oxford Univ. Press},
reportid = {DKFZ-2021-02094},
pages = {526 - 538},
year = {2021},
note = {#LA:B062#},
abstract = {A genetic predisposition to tumor development can be
identified in up to $10\%$ of pediatric patients with
central nervous system (CNS) tumors. For some entities, the
rate of an underlying predisposition is even considerably
higher. In recent years, population-based approaches have
helped to further delineate the role of cancer
predisposition in pediatric oncology. Investigations for
cancer predisposition syndrome (CPS) can be guided by
clinical signs and family history leading to directed
testing of specific genes. The increasingly adopted
molecular analysis of tumor and often parallel blood samples
with multi-gene panel, whole-exome, or whole-genome
sequencing identifies additional patients with or without
clinical signs. Diagnosis of a genetic predisposition may
put an additional burden on affected families. However,
information on a given cancer predisposition may be critical
for the patient as potentially influences treatment
decisions and may offer the patient and healthy carriers the
chance to take part in intensified surveillance programs
aiming at early tumor detection. In this review, we discuss
some of the practical and ethical challenges resulting from
the widespread use of new diagnostic techniques and the most
important CPS that may manifest with brain tumors in
childhood.},
subtyp = {Review Article},
keywords = {cancer (Other) / genetics (Other) / pediatrics (Other) /
predisposition (Other)},
cin = {B062 / HD01},
ddc = {610},
cid = {I:(DE-He78)B062-20160331 / 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:34594567},
pmc = {pmc:PMC8475219},
doi = {10.1093/nop/npab031},
url = {https://inrepo02.dkfz.de/record/172546},
}