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@ARTICLE{Obrecht:163608,
author = {Obrecht, Denise and Mynarek, Martin and Hagel, Christian
and Kwiecien, Robert and Spohn, Michael and Bockmayr,
Michael and Bison, Brigitte and Pfister, Stefan M and Jones,
David T W and Sturm, Dominik and von Deimling, Andreas and
Sahm, Felix and von Hoff, Katja and Juhnke, B-Ole and
Benesch, Martin and Gerber, Nicolas U and Friedrich, Carsten
and von Bueren, André O and Kortmann, Rolf-Dieter and
Schwarz, Rudolf and Pietsch, Torsten and Fleischhack, Gudrun
and Schüller, Ulrich and Rutkowski, Stefan},
title = {{C}linical and molecular characterization of isolated {M}1
disease in pediatric medulloblastoma: experience from the
{G}erman {HIT}-{MED} studies.},
journal = {Journal of neuro-oncology},
volume = {157},
number = {1},
issn = {0167-594X},
address = {Dordrecht [u.a.]},
publisher = {Springer Science + Business Media B.V},
reportid = {DZNE-2022-00354},
pages = {37 - 48},
year = {2022},
note = {(CC BY)},
abstract = {To evaluate the clinical impact of isolated spread of
medulloblastoma cells into cerebrospinal fluid without
additional macroscopic metastases (M1-only).The HIT-MED
database was searched for pediatric patients with M1-only
medulloblastoma diagnosed from 2000 to 2019. Corresponding
clinical and molecular data was evaluated. Treatment was
stratified by age and changed over time for older
patients.70 patients with centrally reviewed M1-only disease
were identified. Clinical data was available for all and
molecular data for 45/70 cases. $91\%$ were non-WNT/non-SHH
medulloblastoma (Grp3/4). 5-year PFS for 52 patients ≥ 4
years was 59.4 (± 7.1) $\%,$ receiving either upfront
craniospinal irradiation (CSI) or SKK-sandwich chemotherapy
(CT). Outcomes did not differ between these strategies
(5-year PFS: CSI 61.7 ± $9.9\%,$ SKK-CT 56.7 ± $6.1\%).$
For patients < 4 years (n = 18), 5-year PFS was 50.0 (±
13.2) $\%.$ M1-persistence occurred exclusively using
postoperative CT and was a strong negative predictive factor
(pPFS/OS < 0.01). Patients with additional clinical or
molecular high-risk (HR) characteristics had worse outcomes
(5-year PFS 42.7 ± $10.6\%$ vs. 64.0 ± $7.0\%,$ p = 0.03).
In n = 22 patients ≥ 4 years with full molecular
information and without additional HR characteristics, risk
classification by molecular subtyping had an effect on
5-year PFS (HR 16.7 ± $15.2\%,$ SR 77.8 ± $13.9\%;$ p =
0.01).Our results confirm that M1-only is a high-risk
condition, and further underline the importance of CSF
staging. Specific risk stratification of affected patients
needs attention in future discussions for trials and
treatment recommendations. Future patients without
contraindications may benefit from upfront CSI by sparing
risks related to higher cumulative CT applied in sandwich
regimen.},
keywords = {Cerebellar Neoplasms: drug therapy / Cerebellar Neoplasms:
therapy / Child / Craniospinal Irradiation / Humans /
Medulloblastoma: drug therapy / Medulloblastoma: therapy /
Risk Factors / Cerebrospinal fluid (Other) / Children
(Other) / Medulloblastoma (Other) / Metastases (Other) /
Radiotherapy (Other)},
cin = {Brainbank Unit Bonn},
ddc = {610},
cid = {I:(DE-2719)1011009},
pnm = {354 - Disease Prevention and Healthy Aging (POF4-354)},
pid = {G:(DE-HGF)POF4-354},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:35190934},
pmc = {pmc:PMC8938370},
doi = {10.1007/s11060-021-03913-5},
url = {https://pub.dzne.de/record/163608},
}