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@ARTICLE{Chavaz:305736,
      author       = {L. Chavaz and A. Bagchi and S. K. Dhanda and F. Toutain and
                      S. M. Pfister$^*$ and D. Sturm$^*$ and T. Pietsch and G. H.
                      Gielen and A. Waha and M. Clarke and C. Lu and M. Karremann
                      and M. Benesch and T. Perwein and G. Nussbaumer and C. Kramm
                      and M. Massimino and V. Biassoni and M. Vinci and A.
                      Mastronuzzi and D. van Vuurden and S. E. M. Veldhuijzen van
                      Zanten and A. Mackay and C. Jones and D. T. W. Jones$^*$ and
                      A. S. Guerreiro Stucklin and U. Tabori and C. Hawkins and S.
                      Ryall and A. Morales La Madrid and A. Lassaletta and S.
                      Bailey and D. Hargrave and J. Chiang and M. El-Ayadi and B.
                      M. Mançano and R. Manuel Reis and C. Hagel and H. Gorsi and
                      N. Silvestrini and A. Gilani and L. Papusha and P. Klimo and
                      X. Zhou and A. Gajjar and G. W. Robinson and A. O. von
                      Bueren},
      title        = {{A} {S}ystematic {S}tudy of {M}olecular {D}iagnosis,
                      {T}reatment and {P}rognosis in {I}nfant-{T}ype {H}emispheric
                      {G}lioma): {A}n {I}ndividual {P}atient {D}ata
                      {M}eta-analysis of 164 patients.},
      journal      = {Neuro-Oncology},
      volume       = {nn},
      issn         = {1522-8517},
      address      = {Oxford},
      publisher    = {Oxford Univ. Press},
      reportid     = {DKFZ-2025-02359},
      pages        = {nn},
      year         = {2025},
      note         = {epub},
      abstract     = {Due 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.},
      keywords     = {Pediatric-type diffuse high-grade glioma (pHGG) (Other) /
                      Receptor Tyrosine Kinase (RTK) (Other) / Tyrosine Kinase
                      Inhibitor (TKI) (Other) / high-grade glioma (HGG) (Other) /
                      infant-type hemispheric glioma (IHG) (Other)},
      cin          = {B062 / B360},
      ddc          = {610},
      cid          = {I:(DE-He78)B062-20160331 / I:(DE-He78)B360-20160331},
      pnm          = {312 - Funktionelle und strukturelle Genomforschung
                      (POF4-312)},
      pid          = {G:(DE-HGF)POF4-312},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:41206756},
      doi          = {10.1093/neuonc/noaf264},
      url          = {https://inrepo02.dkfz.de/record/305736},
}