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@ARTICLE{Tietze:181803,
author = {A. Tietze and K. Mankad and M. H. Lequin and L. Ivarsson
and D. Mirsky and A. Jaju and M. Kool$^*$ and K. V. Hoff and
B. Bison and U. Löbel},
title = {{I}maging {C}haracteristics of {CNS} {N}euroblastoma-
{FOXR}2 : {A} {R}etrospective and {M}ulti-{I}nstitutional
{D}escription of 25 {C}ases},
journal = {American journal of neuroradiology},
volume = {23},
number = {10},
issn = {0195-6108},
address = {Oak Brook, Ill.},
publisher = {Soc.},
reportid = {DKFZ-2022-02223},
pages = {1476-1480},
year = {2022},
note = {2022 Sep 22;43(10):1476-1480},
abstract = {Background and purpose: The 5th edition of the World Health
Organization Classification of CNS tumors defines the CNS
neuroblastoma FOXR2 in the group of embryonal tumors.
Published clinical outcomes tend to suggest a favorable
outcome after resection, craniospinal irradiation, and
chemotherapy. This multicenter study aimed to describe
imaging features of CNS neuroblastoma-FOXR2, which have been
poorly characterized thus far.Materials and methods: On the
basis of a previously published cohort of tumors molecularly
classified as CNS neuroblastoma-FOXR2, patients with
available imaging data were identified. The imaging features
on preoperative MR imaging and CT data were recorded by 8
experienced pediatric neuroradiologists in consensus review
meetings.Results: Twenty-five patients were evaluated (13
girls; median age, 4.5 years). The tumors were often large
(mean, 115 [ SD, 83] mL), showed no $(24\%)$ or limited
$(60\%)$ perilesional edema, demonstrated heterogeneous
enhancement, were often calcified and/or hemorrhagic
$(52\%),$ were always T2WI-hyperintense to GM, and commonly
had cystic and/or necrotic components $(96\%).$ The mean ADC
values were low (687.8 [SD 136.3] × 10-6 mm2/s). The tumors
were always supratentorial. Metastases were infrequent
$(20\%)$ and, when present, were of nodular appearance and
leptomeningeal.Conclusions: In our cohort, CNS neuroblastoma
FOXR2 tumors showed imaging features suggesting high-grade
malignancy and, at the same time, showed characteristics of
less aggressive behavior. There are important differential
diagnoses, but the results of this study may assist in
considering this diagnosis preoperatively.},
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:36137662},
doi = {10.3174/ajnr.A7644},
url = {https://inrepo02.dkfz.de/record/181803},
}