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000277280 1001_ $$0P:(DE-He78)ddb694df5af50ee901e1cd004aa64bfc$$aKolodziejczak, Anna$$b0$$eFirst author$$udkfz
000277280 245__ $$aClinical outcome of pediatric medulloblastoma patients with Li-Fraumeni syndrome.
000277280 260__ $$aOxford$$bOxford Univ. Press$$c2023
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000277280 500__ $$a#EA:B310#LA:B310# / 2023 Dec 8;25(12):2273-2286
000277280 520__ $$aThe prognosis for Li-Fraumeni syndrome (LFS) patients with medulloblastoma (MB) is poor. Comprehensive clinical data for this patient group is lacking, challenging the development of novel therapeutic strategies. Here, we present clinical and molecular data on a retrospective cohort of pediatric LFS MB patients.In this multinational, multicenter retrospective cohort study, LFS patients under 21 years with MB and class 5 or class 4 constitutional TP53 variants were included. TP53 mutation status, methylation subgroup, treatment, progression free- (PFS) and overall survival (OS), recurrence patterns, and incidence of subsequent neoplasms were evaluated.The study evaluated 47 LFS individuals diagnosed with MB, mainly classified as DNA methylation subgroup 'SHH_3' (86%). The majority (74%) of constitutional TP53 variants represented missense variants. The 2- and 5-year (y-) PFS were 36% and 20%, and 2- and 5y-OS were 53% and 23%, respectively. Patients who received post-operative radiotherapy (RT) (2y-PFS: 44%, 2y-OS: 60%) or chemotherapy before RT (2y-PFS: 32%, 2y-OS: 48%) had significantly better clinical outcome then patients who were not treated with RT (2y-PFS: 0%, 2y-OS: 25%). Patients treated according to protocols including high-intensity chemotherapy and patients who received only maintenance-type chemotherapy showed similar outcomes (2y-PFS: 42% and 35%, 2y-OS: 68% and 53%, respectively).LFS MB patients have a dismal prognosis. In the presented cohort use of RT significantly increased survival rates, whereas chemotherapy intensity did not influence their clinical outcome. Prospective collection of clinical data and development of novel treatments are required to improve the outcome of LFS MB patients.
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000277280 650_7 $$2Other$$aLi-Fraumeni syndrome
000277280 650_7 $$2Other$$aTP53
000277280 650_7 $$2Other$$amedulloblastoma
000277280 650_7 $$2Other$$asurvival
000277280 7001_ $$aGuerrini-Rousseau, Lea$$b1
000277280 7001_ $$aPlanchon, Julien Masliah$$b2
000277280 7001_ $$0P:(DE-He78)3de637452ba900e2bdd359b8f41953bf$$aEcker, Jonas$$b3$$udkfz
000277280 7001_ $$0P:(DE-He78)a23e88cc676489fe05be8c178ceaf58e$$aSelt, Florian$$b4$$udkfz
000277280 7001_ $$00000-0003-3302-2719$$aMynarek, Martin$$b5
000277280 7001_ $$aObrecht, Denise$$b6
000277280 7001_ $$0P:(DE-He78)45440b44791309bd4b7dbb4f73333f9b$$aSill, Martin$$b7$$udkfz
000277280 7001_ $$0P:(DE-He78)50c2928009c788a6a5f3a0708dfb6df5$$aAutry, Robert$$b8$$udkfz
000277280 7001_ $$0P:(DE-He78)416cf014d2c21170a2658c31102eb0ad$$aZhao, Eric$$b9$$udkfz
000277280 7001_ $$0P:(DE-He78)5e3d4de4b59cd068969e101847794267$$aHirsch, Steffen$$b10$$udkfz
000277280 7001_ $$aAmouyal, Elsa$$b11
000277280 7001_ $$aDufour, Christelle$$b12
000277280 7001_ $$aAyrault, Olivier$$b13
000277280 7001_ $$aTorrejon, Jacob$$b14
000277280 7001_ $$aWaszak, Sebastian M$$b15
000277280 7001_ $$00000-0002-6557-895X$$aRamaswamy, Vijay$$b16
000277280 7001_ $$aPentikainen, Virve$$b17
000277280 7001_ $$aDemir, Haci Ahmet$$b18
000277280 7001_ $$aClifford, Steven C$$b19
000277280 7001_ $$aSchwalbe, Ed C$$b20
000277280 7001_ $$aMassimi, Luca$$b21
000277280 7001_ $$aSnuderl, Matija$$b22
000277280 7001_ $$00000-0002-5660-866X$$aGalbraith, Kristyn$$b23
000277280 7001_ $$00000-0002-7151-6528$$aKarajannis, Matthias A$$b24
000277280 7001_ $$aHill, Katherine$$b25
000277280 7001_ $$aLi, Bryan K$$b26
000277280 7001_ $$aWalsh, Mike$$b27
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000277280 7001_ $$aBlattmann, Claudia$$b35
000277280 7001_ $$aFilippidou, Maria$$b36
000277280 7001_ $$aPiccolo, Gianluca$$b37
000277280 7001_ $$aScheurlen, Wolfram$$b38
000277280 7001_ $$aFarrag, Ahmed$$b39
000277280 7001_ $$aGrund, Kerstin$$b40
000277280 7001_ $$aSutter, Christian$$b41
000277280 7001_ $$aPietsch, Torsten$$b42
000277280 7001_ $$aFrank, Stephan$$b43
000277280 7001_ $$aSchewe, Denis M$$b44
000277280 7001_ $$aMalkin, David$$b45
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000277280 7001_ $$aLiu, Yen-Lin$$b50
000277280 7001_ $$aCarceller, Fernando$$b51
000277280 7001_ $$aMueller, Sabine$$b52
000277280 7001_ $$00000-0002-1931-7521$$aStoller, Schuyler$$b53
000277280 7001_ $$aTaylor, Michael D$$b54
000277280 7001_ $$00000-0002-5019-2683$$aTabori, Uri$$b55
000277280 7001_ $$00000-0002-6832-6539$$aBouffet, Eric$$b56
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000277280 7001_ $$00000-0002-5669-8546$$avon Hoff, Katja$$b61
000277280 7001_ $$aKratz, Christian P$$b62
000277280 7001_ $$0P:(DE-He78)a46a5b2a871859c8e2d63d2f8c666807$$aSturm, Dominik$$b63$$udkfz
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000277280 7001_ $$0P:(DE-He78)a6b5fcabf661bef95109dbee87dc5271$$avan Tilburg, Cornelis Martinus$$b66$$udkfz
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000277280 7001_ $$00000-0001-9489-6781$$aBourdeaut, Franck$$b71
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