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000305736 1001_ $$aChavaz, Lara$$b0
000305736 245__ $$aA Systematic Study of Molecular Diagnosis, Treatment and Prognosis in Infant-Type Hemispheric Glioma): An Individual Patient Data Meta-analysis of 164 patients.
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000305736 520__ $$aDue to the novelty and rarity of infant-type hemispheric glioma (IHG), optimal treatment and factors determining clinical outcomes are yet to be established.We curated a series of 164 patients with IHG; 155 identified by methodical literature search and nine additional patients contributed by collaborators.All tumors were hemispheric, diagnosed at a median age of 3.4 (0-52) months, and frequently (95%) non-metastatic. 142 (86.5%) tumors harbored fusions involving RTK genes (ALK [67/142, 47%], NTRK1/2/3[32/142, 22.5%], ROS1 [29/142, 20.4%], MET [13/142, 9.2%], and ABL2 [1/142, 0.7%]). 64%, 20%, and 8% of patients were treated with surgery and adjuvant chemotherapy, surgery-only, and surgery plus targeted therapy, respectively. Five patients received radiation. Three-year event-free survival (EFS) and overall survival (OS) was 49.5% [40.7-60.2] and 79.6% [72.1-87.9], respectively. 22 patients succumbed to disease, of which tumor progression (8/22, 36%) and intra-cranial hemorrhage (5/22, 23%) were the most common causes. Multivariate analysis showed that the factors most associated with an increased risk of death were no treatment except for surgery and presence of residual tumor after definitive surgery. These findings present a challenging dichotomy where surgery is both a serious risk factor for early death and, when successful, a benefit.Together, these findings show that IHG is a fusion driven tumor of the very young that is survivable even after progression. While optimal primary therapy for patients with IHG has yet to be established, the findings of this meta-analysis suggest treatment should focus on lowering surgical morbidity and improving its success.
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000305736 650_7 $$2Other$$aPediatric-type diffuse high-grade glioma (pHGG)
000305736 650_7 $$2Other$$aReceptor Tyrosine Kinase (RTK)
000305736 650_7 $$2Other$$aTyrosine Kinase Inhibitor (TKI)
000305736 650_7 $$2Other$$ahigh-grade glioma (HGG)
000305736 650_7 $$2Other$$ainfant-type hemispheric glioma (IHG)
000305736 7001_ $$aBagchi, Aditi$$b1
000305736 7001_ $$aDhanda, Sandeep K$$b2
000305736 7001_ $$aToutain, Fabienne$$b3
000305736 7001_ $$0P:(DE-He78)f746aa965c4e1af518b016de3aaff5d9$$aPfister, Stefan M$$b4$$udkfz
000305736 7001_ $$0P:(DE-He78)a46a5b2a871859c8e2d63d2f8c666807$$aSturm, Dominik$$b5$$udkfz
000305736 7001_ $$aPietsch, Torsten$$b6
000305736 7001_ $$aGielen, Gerrit H$$b7
000305736 7001_ $$aWaha, Andreas$$b8
000305736 7001_ $$aClarke, Matthew$$b9
000305736 7001_ $$aLu, Congyu$$b10
000305736 7001_ $$aKarremann, Michael$$b11
000305736 7001_ $$aBenesch, Martin$$b12
000305736 7001_ $$aPerwein, Thomas$$b13
000305736 7001_ $$aNussbaumer, Gunther$$b14
000305736 7001_ $$aKramm, Christof$$b15
000305736 7001_ $$aMassimino, Maura$$b16
000305736 7001_ $$aBiassoni, Veronica$$b17
000305736 7001_ $$aVinci, Maria$$b18
000305736 7001_ $$aMastronuzzi, Angela$$b19
000305736 7001_ $$avan Vuurden, Dannis$$b20
000305736 7001_ $$aVeldhuijzen van Zanten, Sophie E M$$b21
000305736 7001_ $$aMackay, Alan$$b22
000305736 7001_ $$aJones, Chris$$b23
000305736 7001_ $$0P:(DE-He78)551bb92841f634070997aa168d818492$$aJones, David T W$$b24$$udkfz
000305736 7001_ $$aGuerreiro Stucklin, Ana S$$b25
000305736 7001_ $$aTabori, Uri$$b26
000305736 7001_ $$aHawkins, Cynthia$$b27
000305736 7001_ $$aRyall, Scott$$b28
000305736 7001_ $$aMorales La Madrid, Andrés$$b29
000305736 7001_ $$aLassaletta, Alvaro$$b30
000305736 7001_ $$aBailey, Simon$$b31
000305736 7001_ $$aHargrave, Darren$$b32
000305736 7001_ $$aChiang, Jason$$b33
000305736 7001_ $$aEl-Ayadi, Moatasem$$b34
000305736 7001_ $$aMançano, Bruna Minniti$$b35
000305736 7001_ $$aManuel Reis, Rui$$b36
000305736 7001_ $$aHagel, Christian$$b37
000305736 7001_ $$aGorsi, Hamza$$b38
000305736 7001_ $$aSilvestrini, Nicolas$$b39
000305736 7001_ $$aGilani, Ahmed$$b40
000305736 7001_ $$aPapusha, Ludmilla$$b41
000305736 7001_ $$aKlimo, Paul$$b42
000305736 7001_ $$aZhou, Xin$$b43
000305736 7001_ $$aGajjar, Amar$$b44
000305736 7001_ $$aRobinson, Giles W$$b45
000305736 7001_ $$avon Bueren, Andre O$$b46
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