{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,25]],"date-time":"2025-11-25T07:38:58Z","timestamp":1764056338176,"version":"3.45.0"},"reference-count":31,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2025,11,21]],"date-time":"2025-11-21T00:00:00Z","timestamp":1763683200000},"content-version":"vor","delay-in-days":20,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Cancer Reports"],"published-print":{"date-parts":[[2025,11]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Malignant peripheral nerve sheath tumors (MPNSTs) are rare soft tissue sarcomas with a high risk of recurrence and a poor prognosis, and there is a lack of knowledge regarding long\u2010term follow\u2010up and response to oncological treatment.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Aims<\/jats:title>\n                    <jats:p>The aim of this study was to investigate what treatment the patients received and to examine the outcome for patients with MPNST.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods and Results<\/jats:title>\n                    <jats:p>\n                      This is a retrospective study of patients treated for MPNST at Karolinska University Hospital between 2003 and 2022. Data regarding surgical and oncological treatment and follow\u2010up were collected. Eighty\u2010three patients were identified and included in the study. Tumor grade is available for 72 patients, of which 64 had high\u2010grade tumors. Seventy\u2010nine patients were primarily operated on. Twelve patients presented with distant metastases at diagnosis. Another 37 patients developed local recurrence or distant metastases during follow\u2010up; the median time from surgery to recurrence was 10.5\u2009months (1\u201395\u2009months,\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200936). The overall mortality rate during the study period was 44% (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200982). Twenty\u2010seven patients received palliative systemic treatment. The most used therapy for first\u2010line palliative systemic treatment was doxorubicin and ifosfamide. The disease control rate for first\u2010line treatment was 33% (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200921). The mean overall survival for the cohort was 132\u2009months (95% CI 107\u2013157\u2009months).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Forty\u2010five percent of the patients in this material were diagnosed with recurrent disease and most patients treated with palliative systemic therapy experienced brief disease control following treatment. Among patients with MPNST treated with first\u2010line palliative oncological treatment, doxorubicin and ifosfamide have the highest disease control rates. The study also identified a few patients with long\u2010term treatment responses, with four patients alive more than 2\u2009years after starting palliative oncological treatment.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/cnr2.70406","type":"journal-article","created":{"date-parts":[[2025,11,22]],"date-time":"2025-11-22T04:21:20Z","timestamp":1763785280000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Oncological Outcome for 83 Consecutive Patients With Malignant Peripheral Nerve Sheath Tumors Treated at a Tertiary Referral Centre"],"prefix":"10.1002","volume":"8","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3765-090X","authenticated-orcid":false,"given":"Hannah V. M.","family":"Yeomans","sequence":"first","affiliation":[{"name":"Department of Breast Cancer, Endocrine Tumours and Sarcoma Karolinska University Hospital  Stockholm Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0006-8615-532X","authenticated-orcid":false,"given":"Kira C.","family":"Lloyd","sequence":"additional","affiliation":[{"name":"Department of Breast Cancer, Endocrine Tumours and Sarcoma Karolinska University Hospital  Stockholm Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7015-3841","authenticated-orcid":false,"given":"Felix Haglund","family":"de Flon","sequence":"additional","affiliation":[{"name":"Department of Pathology and Cancer Diagnostics Karolinska University Hospital  Stockholm Sweden"},{"name":"Department of Oncology\u2010Pathology Karolinska Institutet  Stockholm Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0008-7890-7370","authenticated-orcid":false,"given":"Sharmineh","family":"Mansoori","sequence":"additional","affiliation":[{"name":"Department of Medical Sciences Uppsala University  Uppsala Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6631-2053","authenticated-orcid":false,"given":"Panagiotis","family":"Tsagkozis","sequence":"additional","affiliation":[{"name":"Department of Molecular Medicine and Surgery Karolinska Institutet  Stockholm Sweden"},{"name":"Department of Acute and Reparative Medicine Karolinska University Hospital  Stockholm Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0002-1247-7028","authenticated-orcid":false,"given":"Christina M.","family":"Linder Stragliotto","sequence":"additional","affiliation":[{"name":"Department of Breast Cancer, Endocrine Tumours and Sarcoma Karolinska University Hospital  Stockholm Sweden"},{"name":"Department of Molecular Medicine and Surgery Karolinska Institutet  Stockholm Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2025,11,21]]},"reference":[{"key":"e_1_2_12_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.soc.2016.05.009"},{"key":"e_1_2_12_3_1","doi-asserted-by":"publisher","DOI":"10.1093\/jnci\/djx124"},{"key":"e_1_2_12_4_1","doi-asserted-by":"publisher","DOI":"10.1097\/JCMA.0000000000000930"},{"key":"e_1_2_12_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.critrevonc.2019.04.007"},{"key":"e_1_2_12_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11523\u2010024\u201001078\u20105"},{"key":"e_1_2_12_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.humpath.2017.05.010"},{"key":"e_1_2_12_8_1","unstructured":"Confederation of Regional Cancer Centers in Sweden \u201cNational Clinical Cancer Care Guidelines Bone and Soft Tissue Sarcoma [Internet] \u201d 2022 https:\/\/kunskapsbanken.cancercentrum.se\/diagnoser\/sarkom\/vardprogram."},{"issue":"4","key":"e_1_2_12_9_1","first-page":"1957","article-title":"Malignant Peripheral Nerve Sheath Tumors (MPNST) in NF1\u2010Affected Children","volume":"27","author":"Friedrich R. 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