{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,4,24]],"date-time":"2025-04-24T04:34:09Z","timestamp":1745469249996,"version":"3.40.4"},"reference-count":14,"publisher":"S. Karger AG","issue":"2","license":[{"start":{"date-parts":[[2014,1,1]],"date-time":"2014-01-01T00:00:00Z","timestamp":1388534400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"},{"start":{"date-parts":[[2014,1,1]],"date-time":"2014-01-01T00:00:00Z","timestamp":1388534400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Oncology"],"published-print":{"date-parts":[[2014]]},"abstract":"<jats:p>&lt;b&gt;&lt;i&gt;Objective:&lt;\/i&gt;&lt;\/b&gt; Malignant peripheral nerve sheath tumors (MPNST) are a rare subtype of sarcoma, with a poor outcome. MPNST are regarded as being sporadic or associated with neurofibromatosis type 1 (NF1). Few comparative overall-survival (OS) data in these 2 subsets of MPNST patients exist. The aim of this retrospective study was to assess OS in sporadic and NF1-associated MPNST patients. &lt;b&gt;&lt;i&gt;Methods:&lt;\/i&gt;&lt;\/b&gt; Fourteen consecutive patients with initial localized as well as initial metastatic MPNST were diagnosed and treated in our department. Patients with sporadic MPNST were assigned to group A and those with NF1-associated MPNST to group B. &lt;b&gt;&lt;i&gt;Results:&lt;\/i&gt;&lt;\/b&gt; Eight versus 6 patients were allocated to groups A and B. Primary tumors were located on the extremities in all but 1 patient. Two patients in group A and 4 patients in group B experienced a relapse. Four patients died in each of the 2 groups. Median follow-up was 66.2 and 57.2 months in group A and group B, respectively. Median OS in group A was 46.9 months versus 12.7 months in group B. &lt;b&gt;&lt;i&gt;Conclusions:&lt;\/i&gt;&lt;\/b&gt; In this small, single-center study, sporadic-MPNST patients had a longer median OS than those with NF1-associated MPNST.<\/jats:p>","DOI":"10.1159\/000357137","type":"journal-article","created":{"date-parts":[[2014,1,28]],"date-time":"2014-01-28T08:46:40Z","timestamp":1390898800000},"page":"122-126","source":"Crossref","is-referenced-by-count":4,"title":["Clinical Signs of Neurofibromatosis Impact on the Outcome of Malignant Peripheral Nerve Sheath Tumors"],"prefix":"10.1159","volume":"86","author":[{"given":"W.","family":"Lamm","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S.","family":"Schur","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"W.J.","family":"K\u00f6stler","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"P.","family":"Funovics","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R.","family":"Windhager","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"G.","family":"Amann","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J.","family":"Panotopoulos","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"B.","family":"Pokrajac","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"T.","family":"Brodowicz","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"127","published-online":{"date-parts":[[2014,1,28]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1440-1789.2011.01238.x"},{"key":"ref2","doi-asserted-by":"publisher","DOI":"10.1016\/j.suc.2008.04.009"},{"key":"ref3","doi-asserted-by":"publisher","DOI":"10.1245\/s10434-011-1978-7"},{"key":"ref4","doi-asserted-by":"publisher","DOI":"10.1016\/S0360-3016(98)00223-5"},{"key":"ref5","doi-asserted-by":"publisher","DOI":"10.3171\/2012.9.JNS101610"},{"key":"ref6","doi-asserted-by":"publisher","DOI":"10.1016\/j.nec.2008.07.004"},{"key":"ref7","doi-asserted-by":"publisher","DOI":"10.1007\/s11912-009-0045-z"},{"key":"ref8","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.2006.048140"},{"key":"ref9","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.39.5.311"},{"key":"ref10","doi-asserted-by":"publisher","DOI":"10.1155\/2009\/756395"},{"key":"ref11","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.2006.045906"},{"key":"ref12","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.22098"},{"key":"ref13","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejso.2012.09.001"},{"key":"ref14","doi-asserted-by":"publisher","DOI":"10.1093\/neuonc\/nos287"}],"container-title":["Oncology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/357137","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/357137","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/357137","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,23]],"date-time":"2025-04-23T18:25:25Z","timestamp":1745432725000},"score":1,"resource":{"primary":{"URL":"https:\/\/karger.com\/article\/doi\/10.1159\/000357137"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2014]]},"references-count":14,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2014,2,1]]},"published-print":{"date-parts":[[2014,2,11]]}},"URL":"https:\/\/doi.org\/10.1159\/000357137","archive":["Portico"],"relation":{},"ISSN":["0030-2414","1423-0232"],"issn-type":[{"type":"print","value":"0030-2414"},{"type":"electronic","value":"1423-0232"}],"subject":[],"published":{"date-parts":[[2014]]}}}