{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,25]],"date-time":"2026-04-25T04:25:45Z","timestamp":1777091145756,"version":"3.51.4"},"reference-count":29,"publisher":"MDPI AG","issue":"22","license":[{"start":{"date-parts":[[2024,11,7]],"date-time":"2024-11-07T00:00:00Z","timestamp":1730937600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100004581","name":"Ministerium f\u00fcr Wissenschaft, Forschung und Kunst Baden-W\u00fcrttemberg","doi-asserted-by":"publisher","award":["31-7635.41\/215\/2"],"award-info":[{"award-number":["31-7635.41\/215\/2"]}],"id":[{"id":"10.13039\/501100004581","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cancers"],"abstract":"<jats:p>Background\/Objectives: This retrospective observational study aimed to investigate the perioperative outcome in Malignant Peripheral Nerve Sheath Tumors (MPNSTs) with and without relation to Neurofibromatosis Type 1 (NF1) and to detect possible influencing factors. Methods: Clinical reports, histopathological evaluations, imaging, and treatment characteristics were reviewed in 35 operated MPNSTs in 33 patients. Possible predictive valuables included disease type, preoperative tumor volume, SUV and MIB-1 proliferation index, resection margins, the presence of metastasis, and whether radio-\/chemotherapy was received. Results: Patients with NF1 were younger (mean age: 29 \u00b1 13, 8\u201354 years) than sporadic cases (mean age: 45 \u00b1 13, 24\u201367 years) and exhibited significantly larger preoperative tumor volumes (mean 299 vs. 18 cm3, p = 0.048). Most tumors were located in the facial\/cervical\/neck area (34%, n = 12), followed by the trunk (31%, n = 11), lower extremity (17%, n = 6), upper extremity (14%, n = 5), and intraspinal area (3%, n = 1). NF1-associated MPNSTs appeared predominantly on the trunk (39%) and sporadically in the facial\/cervical\/neck area (50%). Complete resection was possible in 66% and an improvement in or stability of function was achieved in most cases (motor 69%, sensory 74%), as well as a decrease in pain intensity (63%). NF1-associated MPNSTs exhibited more severe pain scores (median VRS scale 2, p = 0.002) compared to sporadic tumors (median VRS scale 0.5). Sporadic MPNSTs located at the head\/facial\/brachial plexus and upper extremities exhibited better preoperative functions compared to those on the lower extremities. In 12 cases with available [18F]FDG PET, the mean preoperative SUV (9.8 \u00b1 7.2) positively correlated with the mean maximum MIB-1 index (34 \u00b1 26%, p = 0.005) and the mean preoperative tumor volume (474.7 \u00b1 68.6 cm3, p = 0.047). The overall survival (OS) was significantly longer in tumors with higher resection extents (R0, p = 0.01) and without accompanying metastasis (p = 0.046), and tended to be longer, but not significantly so, in sporadic MPNSTs. In six and seven tumors, with R1\/R2 resection margins and present metastasis, respectively, solid or combined neo-\/adjuvant radio-\/chemotherapy led to a significantly shorter OS (p = 0.014). Conclusions: NF1-associated MPNSTs have larger tumor volumes, higher SUVs and MIB-1 proliferation indices, and a shorter overall survival period. Nevertheless, surgery can improve symptoms, particularly medication-resistant pain, and should also be considered in advanced disease for symptom control\/improvement.<\/jats:p>","DOI":"10.3390\/cancers16223757","type":"journal-article","created":{"date-parts":[[2024,11,7]],"date-time":"2024-11-07T04:22:56Z","timestamp":1730953376000},"page":"3757","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Perioperative Observations and Outcome in Surgical Treatment of Malignant Peripheral Nerve Sheath Tumors"],"prefix":"10.3390","volume":"16","author":[{"given":"Julian","family":"Zipfel","sequence":"first","affiliation":[{"name":"Department of Neurosurgery, University Hospital T\u00fcbingen, BW 72076 T\u00fcbingen, Germany"},{"name":"Centre of Neurofibromatosis and Rare Diseases, University Hospital T\u00fcbingen, BW 72076 T\u00fcbingen, Germany"},{"name":"Section of Pediatric Neurosurgery, University Hospital T\u00fcbingen, BW 72076 T\u00fcbingen, Germany"},{"name":"Division of Pediatric Neurosurgery, BC Children\u2019s Hospital, University of British Columbia, Vancouver, BC V6H 3N1, Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jonas","family":"Tellermann","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, University Hospital T\u00fcbingen, BW 72076 T\u00fcbingen, Germany"},{"name":"Section of Pediatric Neurosurgery, University Hospital T\u00fcbingen, BW 72076 T\u00fcbingen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0919-6658","authenticated-orcid":false,"given":"Kevin Paul","family":"Ferraris","sequence":"additional","affiliation":[{"name":"Division of Pediatric Neurosurgery, BC Children\u2019s Hospital, University of British Columbia, Vancouver, BC V6H 3N1, Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Florian","family":"Grimm","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, University Hospital T\u00fcbingen, BW 72076 T\u00fcbingen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Antje","family":"Bornemann","sequence":"additional","affiliation":[{"name":"Department of Pathology and Neuropathology and Comprehensive Cancer Center, University Hospital T\u00fcbingen, BW 72076 T\u00fcbingen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3205-4631","authenticated-orcid":false,"given":"Benjamin","family":"Bender","sequence":"additional","affiliation":[{"name":"Department of Diagnostic and Interventional Neuroradiology, University Hospital T\u00fcbingen, BW 72076 T\u00fcbingen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Helmut","family":"Dittmann","sequence":"additional","affiliation":[{"name":"Department of Nuclear Medicine and Clinical Molecular Imaging, University Hospital T\u00fcbingen, BW 72076 T\u00fcbingen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J\u00fcrgen","family":"Sch\u00e4fer","sequence":"additional","affiliation":[{"name":"Department of Diagnostic and Interventional Radiology, University Hospital T\u00fcbingen, BW 72076 T\u00fcbingen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Konstantin","family":"Nikolaou","sequence":"additional","affiliation":[{"name":"Department of Diagnostic and Interventional Radiology, University Hospital T\u00fcbingen, BW 72076 T\u00fcbingen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ruth","family":"Ladurner","sequence":"additional","affiliation":[{"name":"Department of General, Visceral and Transplantation Surgery, University Hospital T\u00fcbingen, BW 72076 T\u00fcbingen, Germany"},{"name":"Institute of Clinical Anatomy and Cell Analysis, University of T\u00fcbingen, BW 72076 T\u00fcbingen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Volker","family":"Steger","sequence":"additional","affiliation":[{"name":"Department of Thoracic and Cardiovascular Surgery, German Cardiac Competence Center, University Hospital T\u00fcbingen, BW 72076 T\u00fcbingen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marcos","family":"Tatagiba","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, University Hospital T\u00fcbingen, BW 72076 T\u00fcbingen, Germany"},{"name":"Centre of Neurofibromatosis and Rare Diseases, University Hospital T\u00fcbingen, BW 72076 T\u00fcbingen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Martin U.","family":"Schuhmann","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, University Hospital T\u00fcbingen, BW 72076 T\u00fcbingen, Germany"},{"name":"Centre of Neurofibromatosis and Rare Diseases, University Hospital T\u00fcbingen, BW 72076 T\u00fcbingen, Germany"},{"name":"Section of Pediatric Neurosurgery, University Hospital T\u00fcbingen, BW 72076 T\u00fcbingen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Isabel","family":"Gugel","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, University Hospital T\u00fcbingen, BW 72076 T\u00fcbingen, Germany"},{"name":"Centre of Neurofibromatosis and Rare Diseases, University Hospital T\u00fcbingen, BW 72076 T\u00fcbingen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2024,11,7]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"609","DOI":"10.1007\/s11060-013-1345-6","article-title":"Pediatric and adult malignant peripheral nerve sheath tumors: An analysis of data from the surveillance, epidemiology, and end results program","volume":"116","author":"Amirian","year":"2014","journal-title":"J. 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