{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,8]],"date-time":"2026-10-08T20:06:52Z","timestamp":1791490012290,"version":"4.3.4"},"reference-count":56,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2019,12,30]],"date-time":"2019-12-30T00:00:00Z","timestamp":1577664000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Blood &amp; Cancer"],"published-print":{"date-parts":[[2020,4]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Malignant peripheral nerve sheath tumors (MPNST) are rare and aggressive non\u2010rhabdomyoblastic soft\u2010tissue sarcomas (NRSTS) in children. This study set out to investigate clinical presentation, treatment modalities, and factors associated with survival in pediatric MPNST using Dutch nationwide databases.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Data were obtained from the Netherlands Cancer Registry (NCR) and the Dutch Pathology Database (PALGA) from 1989 to 2017. All primary MPNSTs were collected. Demographic differences were analyzed between adult and pediatric (age \u226418 years) MPNST. In children, demographic and treatment differences between neurofibromatosis type 1 (NF1) and non\u2010NF1 were analyzed. A Cox proportional hazard model was constructed for localized pediatric MPNSTs.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      A total of 70\/784 MPNST patients were children (37.1% NF1). Children did not present differently from adults. In NF1 children, tumor size was more commonly large (&gt;\u00a05\u00a0cm, 92.3% vs 59.1%). Localized disease was primarily resected in 90.6%, and radiotherapy was administered in 37.5%. Non\u2010NF1 children tended to receive chemotherapy more commonly (39.5% vs 26.9%). Overall, estimated five\u2010year survival rates of localized NF1\u2010MPNST was 52.4% (SE: 10.1%) compared with 75.8% (SE: 7.1%) in non\u2010NF1 patients. The multivariate model showed worse survival in NF1 patients (HR: 2.98; 95% CI, 1.17\u20107.60,\n                      <jats:italic>P<\/jats:italic>\n                      \u00a0=\u00a00.02) and increased survival in patients diagnosed after 2005 (HR: 0.20; 95% CI, 0.06\u20100.69,\n                      <jats:italic>P<\/jats:italic>\n                      \u00a0=\u00a00.01). No treatment factors were independently associated with survival.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Pediatric MPNSTs have presentations similar to adult MPNSTs. In children, NF1 patients present with larger tumors, but are treated similarly to non\u2010NF1 MPNSTs. In localized pediatric MPNST, NF1 is associated with worse survival. Promisingly, survival has increased for pediatric MPNSTs after 2005.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/pbc.28138","type":"journal-article","created":{"date-parts":[[2019,12,31]],"date-time":"2019-12-31T02:45:18Z","timestamp":1577760318000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":35,"title":["Neurofibromatosis\u2010associated malignant peripheral nerve sheath tumors in children have a worse prognosis: A nationwide cohort study"],"prefix":"10.1002","volume":"67","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8678-9203","authenticated-orcid":false,"given":"Enrico","family":"Martin","sequence":"first","affiliation":[{"name":"Department of Plastic and Reconstructive Surgery University Medical Center Utrecht  Utrecht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J. Henk","family":"Coert","sequence":"additional","affiliation":[{"name":"Department of Plastic and Reconstructive Surgery University Medical Center Utrecht  Utrecht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Uta E.","family":"Flucke","sequence":"additional","affiliation":[{"name":"Department of Pathology University Medical Center Utrecht  Utrecht The Netherlands"},{"name":"Department of Pathology Radboud University Medical Center  Nijmegen The Netherlands"},{"name":"Diagnostic Laboratory and Pathology Princess M\u00e1xima Center for Pediatric Oncology  Utrecht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Willem\u2010Bart M.","family":"Slooff","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery University Medical Center Utrecht  Utrecht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michiel A.J.","family":"van de Sande","sequence":"additional","affiliation":[{"name":"Department of Orthopedic Surgery Leiden University Medical Center  Leiden The Netherlands"},{"name":"Department of Solid Tumors Princess M\u00e1xima Center for Pediatric Oncology  Utrecht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0503-5838","authenticated-orcid":false,"given":"Max M.","family":"van Noesel","sequence":"additional","affiliation":[{"name":"Department of Solid Tumors Princess M\u00e1xima Center for Pediatric Oncology  Utrecht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dirk J.","family":"Gr\u00fcnhagen","sequence":"additional","affiliation":[{"name":"Department of Surgical Oncology Erasmus Medical Center Cancer Institute  Rotterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marc H.W.A.","family":"Wijnen","sequence":"additional","affiliation":[{"name":"Department of Solid Tumors Princess M\u00e1xima Center for Pediatric Oncology  Utrecht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cornelis","family":"Verhoef","sequence":"additional","affiliation":[{"name":"Department of Surgical Oncology Erasmus Medical Center Cancer Institute  Rotterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2019,12,30]]},"reference":[{"issue":"6","key":"e_1_2_10_2_1","first-page":"2597","article-title":"Incidence and survival in sarcoma in the United States: a focus on musculoskeletal lesions","volume":"33","author":"Ng VY","year":"2013","journal-title":"Anticancer Res"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1097\/SLA.0b013e3181a77e9a"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1245\/s10434-011-1978-7"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1093\/nop\/npy025"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.25149"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2005.01.4886"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.68.3.353"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00256-016-2394-7"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(19850601)55:11<2543::AID-CNCR2820551105>3.0.CO;2-4"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.3892\/ol.2015.3762"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejca.2015.12.015"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-911X(199804)30:4<201::AID-MPO1>3.0.CO;2-K"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2002.06.066"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2005.02.053"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.27833"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejca.2010.11.013"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.3389\/fonc.2014.00324"},{"key":"e_1_2_10_19_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11864-015-0328-6"},{"key":"e_1_2_10_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijrobp.2015.02.007"},{"key":"e_1_2_10_21_1","doi-asserted-by":"publisher","DOI":"10.1093\/neuonc\/nos287"},{"key":"e_1_2_10_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejca.2019.10.014"},{"issue":"1","key":"e_1_2_10_23_1","first-page":"19","article-title":"Pathology databanking and biobanking in The Netherlands, a central role for PALGA, the nationwide histopathology and cytopathology data network and archive","volume":"29","author":"Casparie M","year":"2007","journal-title":"Cell Oncol"},{"key":"e_1_2_10_24_1","unstructured":"TherneauTM CrowsonCS ElizabethAJ. 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