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@ARTICLE{Kommoss:274458,
      author       = {F. K. F. Kommoss and A.-S. Chong and A.-L. Chong and E.
                      Pfaff$^*$ and D. Jones$^*$ and L. S. Hiemcke-Jiwa and L. A.
                      Kester and U. Flucke and M. Gessler and D. Schrimpf$^*$ and
                      F. Sahm$^*$ and B. A. Clarke and C. J. R. Stewart and Y.
                      Wang and C. B. Gilks and F. Kommoss and D. G. Huntsman and
                      U. Schüller and C. Koelsche and W. Glenn McCluggage and A.
                      von Deimling$^*$ and W. D. Foulkes},
      title        = {{G}enomic characterization of {DICER}1-associated neoplasms
                      uncovers molecular classes.},
      journal      = {Nature Communications},
      volume       = {14},
      number       = {1},
      issn         = {2041-1723},
      address      = {[London]},
      publisher    = {Nature Publishing Group UK},
      reportid     = {DKFZ-2023-00603},
      pages        = {1677},
      year         = {2023},
      note         = {#LA:B300#},
      abstract     = {DICER1 syndrome is a tumor predisposition syndrome that is
                      associated with up to 30 different neoplastic lesions,
                      usually affecting children and adolescents. Here we identify
                      a group of mesenchymal tumors which is highly associated
                      with DICER1 syndrome, and molecularly distinct from other
                      DICER1-associated tumors. This group of DICER1-associated
                      mesenchymal tumors encompasses multiple well-established
                      clinicopathological tumor entities and can be further
                      divided into three clinically meaningful classes designated
                      'low-grade mesenchymal tumor with DICER1 alteration' (LGMT
                      DICER1), 'sarcoma with DICER1 alteration' (SARC DICER1), and
                      primary intracranial sarcoma with DICER1 alteration (PIS
                      DICER1). Our study not only provides a combined approach to
                      classify DICER1-associated neoplasms for improved clinical
                      management but also suggests a role for global
                      hypomethylation and other recurrent molecular events in
                      sarcomatous differentiation in mesenchymal tumors with
                      DICER1 alteration. Our results will facilitate future
                      investigations into prognostication and therapeutic
                      approaches for affected patients.},
      cin          = {B360 / B300 / HD01},
      ddc          = {500},
      cid          = {I:(DE-He78)B360-20160331 / I:(DE-He78)B300-20160331 /
                      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:36966138},
      doi          = {10.1038/s41467-023-37092-w},
      url          = {https://inrepo02.dkfz.de/record/274458},
}