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@ARTICLE{MontielEquihua:309872,
      author       = {C. Montiel Equihua and J. J. Molenaar and I. Areso and J.
                      A. Biegel and P. Blanc and S. N. Chi and S. Daems and L.
                      Danielson and J. Drost and N. E. Franke and M. C. Frühwald
                      and A. Gajjar and J. I. Geller and A. Huang and P. D. Johann
                      and P. Kearns and K. Nysom and S. O'Connor and M. V. Ortiz
                      and J. Parker and S. Patel and S. Patel and C. W. Roberts
                      and D. Williamson and J. S. Yi and A. D. Pearson and D.
                      Jenkinson and M. Kool$^*$ and F. Bourdeaut},
      collaboration = {LifeArc and I. T. f. C. w. Cancer and C. R. UK and C. G. C.
                      P. team},
      title        = {{P}aediatric {T}herapeutic {D}evelopment {W}orkshop on
                      rhabdoid tumours.},
      journal      = {British journal of cancer},
      volume       = {nn},
      issn         = {0007-0920},
      address      = {Edinburgh},
      publisher    = {Nature Publ. Group},
      reportid     = {DKFZ-2026-00357},
      pages        = {nn},
      year         = {2026},
      note         = {#DKTKZFB26# / epub},
      abstract     = {Rhabdoid tumours (RT) are malignancies of the central
                      nervous system, kidneys, liver and soft tissues that most
                      commonly affect very young children with survival rates
                      below $30\%$ in high-risk cohorts. Treatment entails
                      surgery, intensive chemotherapy and radiotherapy, associated
                      with substantial short- and long-term toxicities. There is
                      an unmet need to develop targeted therapies for RT to
                      improve patient outcomes and mitigate the toxicities of
                      current therapy. Detailed research followed by a workshop
                      had the objective of enabling the development of targeted
                      therapeutics for RT. Given the inherent commonality of their
                      biology (i.e. biallelic inactivation of SMARCB1 or more
                      rarely SMARCA4) the therapeutic approach should be similar
                      for intra-cranial and extra-cranial tumours.
                      DDB1-CUL4-associated factor 5 is a promising target, and the
                      development of small molecule binders/degraders is a
                      priority. Enhancer of zeste 2 polycomb repressive complex 2
                      subunit (EZH2) degraders may have greater therapeutic
                      potential than inhibitors. Fibroblast growth factor receptor
                      and platelet-derived growth factor receptor inhibitors may
                      have value in subgroups. Mouse double minute 2 homologue
                      (MDM2) is a priority target for novel therapeutic
                      development and combination trials. Combinations of EZH2,
                      MDM2 inhibitors and selective inhibitors of nuclear export
                      should be evaluated robustly preclinically and drive early
                      clinical studies.},
      subtyp        = {Review Article},
      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:41680284},
      doi          = {10.1038/s41416-026-03348-7},
      url          = {https://inrepo02.dkfz.de/record/309872},
}