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@ARTICLE{Upadhyaya:168085,
      author       = {S. A. Upadhyaya and G. Robinson and A. Onar-Thomas and B.
                      A. Orr and P. Johann and G. Wu and C. A. Billups and R. G.
                      Tatevossian and S. K. Dhanda and A. Srinivasan and A.
                      Broniscer and I. Qaddoumi and A. Vinitsky and G. T.
                      Armstrong and A. Bendel and T. E. Hassall and S. Partap and
                      P. G. Fisher and J. R. Crawford and M. M. Chintagumpala and
                      E. Bouffet and S. Gururangan and R. Mostafavi and R. P.
                      Sanders and P. Klimo and Z. Patay and D. J. Indelicato and
                      K. E. Nichols and F. A. Boop and T. E. Merchant and M.
                      Kool$^*$ and D. W. Ellison and A. Gajjar},
      title        = {{R}elevance of {M}olecular {G}roups in {C}hildren with
                      {N}ewly {D}iagnosed {A}typical {T}eratoid {R}habdoid
                      {T}umor: {R}esults from {P}rospective {S}t. {J}ude
                      {M}ulti-{I}nstitutional {T}rials.},
      journal      = {Clinical cancer research},
      volume       = {27},
      number       = {10},
      issn         = {1557-3265},
      address      = {Philadelphia, Pa. [u.a.]},
      publisher    = {AACR},
      reportid     = {DKFZ-2021-00673},
      pages        = {2879-2889},
      year         = {2021},
      note         = {27(10):2879-2889},
      abstract     = {Report relevance of molecular groups to clinico-pathologic
                      features, germline SMARCB1/SMARCA4 alterations (GLA), and
                      survival of children with atypical teratoid rhabdoid tumor
                      (ATRT) treated in two multi-institutional clinical
                      trials.Seventy-four participants with newly diagnosed ATRT
                      were treated in two trials: infants (SJYC07: age<3 years;
                      n=52) and children (SJMB03: age 3-21 years; n=22), using
                      surgery, conventional chemotherapy (infants), or dose-dense
                      chemotherapy with autologous stem cell rescue (children),
                      and age- and risk-adapted radiation therapy [focal (infants)
                      and craniospinal (CSI) (children)]. Molecular groups
                      ATRT-MYC (MYC), ATRT-SHH (SHH), and ATRT-TYR (TYR) were
                      determined from tumor DNA methylation profiles.Twenty-four
                      participants $(32\%)$ were alive at time of analysis at a
                      median follow-up of 8.4 years (range, 3.1-14.1 years).
                      Methylation profiling classified 64 ATRTs as TYR (n=21), SHH
                      (n=30), and MYC (n=13), SHH group being associated with
                      metastatic disease. Among infants, TYR group had the best
                      overall survival (OS) (P=0.02). However, outcomes did not
                      differ by molecular groups among infants with non-metastatic
                      (M0) disease. Children with M0 disease and <1.5 cm2 residual
                      tumor had a 5-year progression-free survival (PFS) of
                      72.7{plus $minus}12.7\%$ and OS of 81.8{plus $minus}11\%.$
                      Infants with M0 disease had a 5-year PFS of 39.1{plus
                      $minus}11.5\%$ and OS of 51.8{plus $minus}12\%.$ Those with
                      metastases fared poorly [5-year OS 25{plus $minus}12.5\%$
                      (children) and $0\%$ (infants)]. SMARCB1 GLAs were not
                      associated with PFS.Among infants, those with ATRT-TYR had
                      the best OS. ATRT-SHH was associated with metastases and
                      consequently with inferior outcomes. Children with
                      non-metastatic ATRT benefit from post-operative CSI and
                      adjuvant chemotherapy.},
      cin          = {B062},
      ddc          = {610},
      cid          = {I:(DE-He78)B062-20160331},
      pnm          = {312 - Funktionelle und strukturelle Genomforschung
                      (POF4-312)},
      pid          = {G:(DE-HGF)POF4-312},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:33737307},
      doi          = {10.1158/1078-0432.CCR-20-4731},
      url          = {https://inrepo02.dkfz.de/record/168085},
}