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@ARTICLE{Barthelme:119617,
      author       = {S. Barthelmeß and H. Geddert and C. Boltze and E. A.
                      Moskalev and M. Bieg$^*$ and H. Sirbu and B. Brors$^*$ and
                      S. Wiemann$^*$ and A. Hartmann and A. Agaimy and F. Haller},
      title        = {{S}olitary fibrous tumors/hemangiopericytomas with
                      different variants of the {NAB}2-{STAT}6 gene fusion are
                      characterized by specific histomorphology and distinct
                      clinicopathological features.},
      journal      = {The American journal of pathology},
      volume       = {184},
      number       = {4},
      issn         = {0002-9440},
      address      = {New York [u.a.]},
      publisher    = {Elsevier},
      reportid     = {DKFZ-2017-00248},
      pages        = {1209 - 1218},
      year         = {2014},
      abstract     = {Recurrent somatic fusions of the two genes, NGFI-A-binding
                      protein 2 (NAB2) and STAT6, located at chromosomal region
                      12q13, have been recently identified to be presumable
                      tumor-initiating events in solitary fibrous tumors (SFT).
                      Herein, we evaluated a cohort of 52 SFTs/hemangiopericytomas
                      (HPCs) by whole-exome sequencing (one case) and multiplex
                      RT-PCR (all 52 cases), and identified 12 different
                      NAB2-STAT6 fusion variants in 48 cases $(92\%).$ All 52
                      cases showed strong and diffuse nuclear positivity for STAT6
                      by IHC. We categorized the fusion variants according to
                      their potential functional effects within the predicted
                      fusion protein and found strong correlations with relevant
                      clinicopathological features. Tumors with the most common
                      fusion variant, NAB2ex4-STAT6ex2/3, corresponded to classic
                      pleuropulmonary SFTs with diffuse fibrosis and mostly benign
                      behavior and occurred in older patients (median age, 69
                      years). In contrast, tumors with the second most common
                      fusion variant, NAB2ex6-STAT6ex16/17, were found in much
                      younger patients (median age, 47 years) and represented
                      typical HPCs from deep soft tissue with a more aggressive
                      phenotype and clinical behavior. In summary, these molecular
                      genetic findings support the concept that classic
                      pleuropulmonary SFT and deep-seated HPC are separate
                      entities that share common features but correlate to
                      different clinical outcome.},
      keywords     = {NAB2 protein, human (NLM Chemicals) / Repressor Proteins
                      (NLM Chemicals) / STAT6 Transcription Factor (NLM Chemicals)
                      / STAT6 protein, human (NLM Chemicals)},
      cin          = {B080 / B050 / W110},
      ddc          = {610},
      cid          = {I:(DE-He78)B080-20160331 / I:(DE-He78)B050-20160331 /
                      I:(DE-He78)W110-20160331},
      pnm          = {312 - Functional and structural genomics (POF3-312)},
      pid          = {G:(DE-HGF)POF3-312},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:24513261},
      doi          = {10.1016/j.ajpath.2013.12.016},
      url          = {https://inrepo02.dkfz.de/record/119617},
}