000119617 001__ 119617 000119617 005__ 20240228134945.0 000119617 0247_ $$2doi$$a10.1016/j.ajpath.2013.12.016 000119617 0247_ $$2pmid$$apmid:24513261 000119617 0247_ $$2ISSN$$a0002-9440 000119617 0247_ $$2ISSN$$a1525-2191 000119617 037__ $$aDKFZ-2017-00248 000119617 041__ $$aeng 000119617 082__ $$a610 000119617 1001_ $$aBarthelmeß, Sarah$$b0 000119617 245__ $$aSolitary fibrous tumors/hemangiopericytomas with different variants of the NAB2-STAT6 gene fusion are characterized by specific histomorphology and distinct clinicopathological features. 000119617 260__ $$aNew York [u.a.]$$bElsevier$$c2014 000119617 3367_ $$2DRIVER$$aarticle 000119617 3367_ $$2DataCite$$aOutput Types/Journal article 000119617 3367_ $$0PUB:(DE-HGF)16$$2PUB:(DE-HGF)$$aJournal Article$$bjournal$$mjournal$$s1520584871_26905 000119617 3367_ $$2BibTeX$$aARTICLE 000119617 3367_ $$2ORCID$$aJOURNAL_ARTICLE 000119617 3367_ $$00$$2EndNote$$aJournal Article 000119617 520__ $$aRecurrent somatic fusions of the two genes, NGFI-A-binding protein 2 (NAB2) and STAT6, located at chromosomal region 12q13, have been recently identified to be presumable tumor-initiating events in solitary fibrous tumors (SFT). Herein, we evaluated a cohort of 52 SFTs/hemangiopericytomas (HPCs) by whole-exome sequencing (one case) and multiplex RT-PCR (all 52 cases), and identified 12 different NAB2-STAT6 fusion variants in 48 cases (92%). All 52 cases showed strong and diffuse nuclear positivity for STAT6 by IHC. We categorized the fusion variants according to their potential functional effects within the predicted fusion protein and found strong correlations with relevant clinicopathological features. Tumors with the most common fusion variant, NAB2ex4-STAT6ex2/3, corresponded to classic pleuropulmonary SFTs with diffuse fibrosis and mostly benign behavior and occurred in older patients (median age, 69 years). In contrast, tumors with the second most common fusion variant, NAB2ex6-STAT6ex16/17, were found in much younger patients (median age, 47 years) and represented typical HPCs from deep soft tissue with a more aggressive phenotype and clinical behavior. In summary, these molecular genetic findings support the concept that classic pleuropulmonary SFT and deep-seated HPC are separate entities that share common features but correlate to different clinical outcome. 000119617 536__ $$0G:(DE-HGF)POF3-312$$a312 - Functional and structural genomics (POF3-312)$$cPOF3-312$$fPOF III$$x0 000119617 588__ $$aDataset connected to CrossRef, PubMed, 000119617 650_7 $$2NLM Chemicals$$aNAB2 protein, human 000119617 650_7 $$2NLM Chemicals$$aRepressor Proteins 000119617 650_7 $$2NLM Chemicals$$aSTAT6 Transcription Factor 000119617 650_7 $$2NLM Chemicals$$aSTAT6 protein, human 000119617 7001_ $$aGeddert, Helene$$b1 000119617 7001_ $$aBoltze, Carsten$$b2 000119617 7001_ $$aMoskalev, Evgeny A$$b3 000119617 7001_ $$0P:(DE-He78)e247efc0264a7b0e4bae09804002d4e1$$aBieg, Matthias$$b4$$udkfz 000119617 7001_ $$aSirbu, Horia$$b5 000119617 7001_ $$0P:(DE-He78)fc949170377b58098e46141d95c72661$$aBrors, Benedikt$$b6$$udkfz 000119617 7001_ $$0P:(DE-He78)f6bebe05e7a748d3cbf9f59659567d52$$aWiemann, Stefan$$b7$$udkfz 000119617 7001_ $$aHartmann, Arndt$$b8 000119617 7001_ $$aAgaimy, Abbas$$b9 000119617 7001_ $$aHaller, Florian$$b10 000119617 773__ $$0PERI:(DE-600)1480207-7$$a10.1016/j.ajpath.2013.12.016$$gVol. 184, no. 4, p. 1209 - 1218$$n4$$p1209 - 1218$$tThe American journal of pathology$$v184$$x0002-9440$$y2014 000119617 909CO $$ooai:inrepo02.dkfz.de:119617$$pVDB 000119617 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)e247efc0264a7b0e4bae09804002d4e1$$aDeutsches Krebsforschungszentrum$$b4$$kDKFZ 000119617 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)fc949170377b58098e46141d95c72661$$aDeutsches Krebsforschungszentrum$$b6$$kDKFZ 000119617 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)f6bebe05e7a748d3cbf9f59659567d52$$aDeutsches Krebsforschungszentrum$$b7$$kDKFZ 000119617 9131_ $$0G:(DE-HGF)POF3-312$$1G:(DE-HGF)POF3-310$$2G:(DE-HGF)POF3-300$$3G:(DE-HGF)POF3$$4G:(DE-HGF)POF$$aDE-HGF$$bGesundheit$$lKrebsforschung$$vFunctional and structural genomics$$x0 000119617 9141_ $$y2014 000119617 915__ $$0StatID:(DE-HGF)0100$$2StatID$$aJCR$$bAM J PATHOL : 2015 000119617 915__ $$0StatID:(DE-HGF)0200$$2StatID$$aDBCoverage$$bSCOPUS 000119617 915__ $$0StatID:(DE-HGF)0300$$2StatID$$aDBCoverage$$bMedline 000119617 915__ $$0StatID:(DE-HGF)0310$$2StatID$$aDBCoverage$$bNCBI Molecular Biology Database 000119617 915__ $$0StatID:(DE-HGF)0199$$2StatID$$aDBCoverage$$bThomson Reuters Master Journal List 000119617 915__ $$0StatID:(DE-HGF)0110$$2StatID$$aWoS$$bScience Citation Index 000119617 915__ $$0StatID:(DE-HGF)0150$$2StatID$$aDBCoverage$$bWeb of Science Core Collection 000119617 915__ $$0StatID:(DE-HGF)0111$$2StatID$$aWoS$$bScience Citation Index Expanded 000119617 915__ $$0StatID:(DE-HGF)1110$$2StatID$$aDBCoverage$$bCurrent Contents - Clinical Medicine 000119617 915__ $$0StatID:(DE-HGF)1030$$2StatID$$aDBCoverage$$bCurrent Contents - Life Sciences 000119617 915__ $$0StatID:(DE-HGF)1050$$2StatID$$aDBCoverage$$bBIOSIS Previews 000119617 915__ $$0StatID:(DE-HGF)9900$$2StatID$$aIF < 5 000119617 9201_ $$0I:(DE-He78)B080-20160331$$kB080$$lTheoretische Bioinformatik$$x0 000119617 9201_ $$0I:(DE-He78)B050-20160331$$kB050$$lMolekulare Genomanalyse$$x1 000119617 9201_ $$0I:(DE-He78)W110-20160331$$kW110$$lMicroarray Unit$$x2 000119617 980__ $$ajournal 000119617 980__ $$aVDB 000119617 980__ $$aI:(DE-He78)B080-20160331 000119617 980__ $$aI:(DE-He78)B050-20160331 000119617 980__ $$aI:(DE-He78)W110-20160331 000119617 980__ $$aUNRESTRICTED