{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,21]],"date-time":"2026-09-21T14:02:52Z","timestamp":1789999372671,"version":"4.0.1"},"reference-count":38,"publisher":"MDPI AG","issue":"18","license":[{"start":{"date-parts":[[2026,9,20]],"date-time":"2026-09-20T00:00:00Z","timestamp":1789862400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cancers"],"abstract":"<jats:p>Objective: Hybrid peripheral nerve sheath tumors (HPNSTs) are rare benign neoplasms combining features of more than one peripheral nerve sheath tumor subtype\u2014most commonly schwannoma, neurofibroma, and\/or perineurioma. Multicenter registry data were used to improve their clinical, radiological, histopathological, and syndromic characterization. Methods: This study analyzed 55 histologically confirmed HPNSTs in 52 patients from a multicenter registry of 627 surgically treated patients at four tertiary neurosurgical centers in Germany and Austria. Demographic, anatomical, clinical, imaging, histopathological, surgical, follow-up, and syndrome-associated variables were evaluated. Results: HPNSTs were identified in 52\/627 surgically treated registry patients (8.3%), comprising 55 lesions. Median age at surgery was 57 years (IQR 44\u201363 years; range, 18\u201378), and 27\/52 patients (51.9%) were female. Lower-extremity or lumbosacral lesions were more common than upper-extremity or cervicothoracic lesions (32 vs. 23 cases). Schwannoma\/neurofibroma hybrid was the predominant subtype (51\/55, 92.7%), followed by schwannoma\/perineurioma and triphasic schwannoma\/neurofibroma\/perineurioma tumors (2\/55 each, 3.6%). All tumors were benign. Provoked pain was the leading symptom (47\/55, 85.5%), whereas motor deficits were uncommon (5\/55, 9.1%). MRI frequently showed inhomogeneous contrast enhancement (42\/48, 87.5%) and cystic components (19\/47, 40.4%). Complete resection was achieved in 49\/55 tumors (89.1%). A documented or suspected syndromic association was present in 18\/52 patients (34.6%). Conclusions: In this surgically selected multicenter registry cohort, HPNSTs accounted for 8.3% of operated peripheral nerve tumor patients. This cohort frequency should not be interpreted as population prevalence. The integrated clinical, imaging, pathological, and surgical data highlight pain burden, multifocality, and potential syndromic associations. Long-term behavior cannot be determined because follow-up was short and incomplete.<\/jats:p>","DOI":"10.3390\/cancers18183053","type":"journal-article","created":{"date-parts":[[2026,9,21]],"date-time":"2026-09-21T12:50:03Z","timestamp":1789995003000},"page":"3053","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Contemporary Profile of Hybrid Nerve Sheath Tumors: Clinical, Anatomical, and Imaging Characteristics"],"prefix":"10.3390","volume":"18","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7673-0179","authenticated-orcid":false,"given":"Nadja","family":"Gr\u00fcbel","sequence":"first","affiliation":[{"name":"Peripheral Nerve Unit, Department of Neurosurgery, University of Ulm, Lindenallee 2, 89312 G\u00fcnzburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"},{"role":"conceptualization","vocabulary":"credit"},{"role":"formal-analysis","vocabulary":"credit"},{"role":"writing-original-draft","vocabulary":"credit"},{"role":"project-administration","vocabulary":"credit"},{"role":"resources","vocabulary":"credit"},{"role":"data-curation","vocabulary":"credit"},{"role":"supervision","vocabulary":"credit"},{"role":"methodology","vocabulary":"credit"},{"role":"investigation","vocabulary":"credit"},{"role":"visualization","vocabulary":"credit"}]},{"given":"Anne-Kathrin","family":"Uerschels","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, University of Essen, Hufelandstra\u00dfe 55, 45147 Essen, Germany"},{"name":"Department of Neurosurgery, Gemeinschaftsklinikum Mittelrhein, Johannes-M\u00fcller-Stra\u00dfe 7, 56068 Koblenz, Germany"}],"role":[{"vocabulary":"crossref","role":"author"},{"role":"writing-review-editing","vocabulary":"credit"},{"role":"data-curation","vocabulary":"credit"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0054-1611","authenticated-orcid":false,"given":"Oliver","family":"Gembruch","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, University of Essen, Hufelandstra\u00dfe 55, 45147 Essen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"},{"role":"writing-review-editing","vocabulary":"credit"},{"role":"data-curation","vocabulary":"credit"}]},{"given":"Karsten","family":"Wrede","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, University of Essen, Hufelandstra\u00dfe 55, 45147 Essen, Germany"},{"name":"Department of Neurosurgery, Gemeinschaftsklinikum Mittelrhein, Johannes-M\u00fcller-Stra\u00dfe 7, 56068 Koblenz, Germany"}],"role":[{"vocabulary":"crossref","role":"author"},{"role":"writing-review-editing","vocabulary":"credit"},{"role":"data-curation","vocabulary":"credit"}]},{"given":"Felix","family":"Sch\u00f6ffing","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Medical Center-University of Freiburg, Faculty of Medicine, University of Freiburg, 79106 Freiburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"},{"role":"writing-review-editing","vocabulary":"credit"},{"role":"data-curation","vocabulary":"credit"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8743-3161","authenticated-orcid":false,"given":"Marc","family":"Hohenhaus","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Medical Center-University of Freiburg, Faculty of Medicine, University of Freiburg, 79106 Freiburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"},{"role":"writing-review-editing","vocabulary":"credit"},{"role":"data-curation","vocabulary":"credit"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1469-2901","authenticated-orcid":false,"given":"Christoph","family":"Scholz","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Medical Center-University of Freiburg, Faculty of Medicine, University of Freiburg, 79106 Freiburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"},{"role":"writing-review-editing","vocabulary":"credit"},{"role":"data-curation","vocabulary":"credit"}]},{"given":"Katharina","family":"Hobitsch","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery & Neurorestoration, Klinikum Klagenfurt, 9020 Klagenfurt, Carinthia, Austria"}],"role":[{"vocabulary":"crossref","role":"author"},{"role":"writing-review-editing","vocabulary":"credit"},{"role":"data-curation","vocabulary":"credit"}]},{"given":"Thomas","family":"Kretschmer","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery & Neurorestoration, Klinikum Klagenfurt, 9020 Klagenfurt, Carinthia, Austria"}],"role":[{"vocabulary":"crossref","role":"author"},{"role":"writing-review-editing","vocabulary":"credit"},{"role":"data-curation","vocabulary":"credit"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2133-714X","authenticated-orcid":false,"given":"Simone","family":"D\u2019Souza","sequence":"additional","affiliation":[{"name":"Epilepsy Center Frankfurt Rhine-Main, Department of Neurology, University Medical Center Frankfurt, Goethe-University Frankfurt, 60596 Frankfurt, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7783-8053","authenticated-orcid":false,"given":"Nora F.","family":"Dengler","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Helios Hospital Bad Saarow, Pieskower Str. 33, 15526 Bad Saarow, Germany"}],"role":[{"vocabulary":"crossref","role":"author"},{"role":"writing-review-editing","vocabulary":"credit"},{"role":"conceptualization","vocabulary":"credit"}]},{"given":"Christian Rainer","family":"Wirtz","sequence":"additional","affiliation":[{"name":"Peripheral Nerve Unit, Department of Neurosurgery, University of Ulm, Lindenallee 2, 89312 G\u00fcnzburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"},{"role":"writing-review-editing","vocabulary":"credit"},{"role":"data-curation","vocabulary":"credit"}]},{"given":"Stefanie","family":"Deininger","sequence":"additional","affiliation":[{"name":"Peripheral Nerve Unit, Department of Neurosurgery, University of Ulm, Lindenallee 2, 89312 G\u00fcnzburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"},{"role":"writing-review-editing","vocabulary":"credit"},{"role":"data-curation","vocabulary":"credit"}]},{"given":"Anja","family":"Osterloh","sequence":"additional","affiliation":[{"name":"Institute of Neuropathology, University Hospital Ulm, Albert-Einstein-Allee 23, 89081 Ulm, Germany"}],"role":[{"vocabulary":"crossref","role":"author"},{"role":"writing-review-editing","vocabulary":"credit"},{"role":"data-curation","vocabulary":"credit"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0268-9425","authenticated-orcid":false,"given":"Juliane","family":"Bremer","sequence":"additional","affiliation":[{"name":"Institute of Neuropathology, University Hospital RWTH Aachen, Pauwelstra\u00dfe 19, 52074 Aachen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"},{"role":"writing-original-draft","vocabulary":"credit"},{"role":"data-curation","vocabulary":"credit"},{"role":"resources","vocabulary":"credit"}]},{"given":"Maria Teresa","family":"Pedro","sequence":"additional","affiliation":[{"name":"Peripheral Nerve Unit, Department of Neurosurgery, University of Ulm, Lindenallee 2, 89312 G\u00fcnzburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"},{"role":"conceptualization","vocabulary":"credit"},{"role":"methodology","vocabulary":"credit"},{"role":"writing-original-draft","vocabulary":"credit"},{"role":"data-curation","vocabulary":"credit"},{"role":"validation","vocabulary":"credit"},{"role":"supervision","vocabulary":"credit"}]},{"name":"on behalf of the Peripheral Nerve Tumor Study (PNTR) Study Group","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,9,20]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"405","DOI":"10.1046\/j.1365-2559.1998.00419.x","article-title":"Nerve Sheath Tumours with Hybrid Features of Neurofibroma and Schwannoma: A Conceptual 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(2025). Hybrid Benign Peripheral Nerve Sheath Tumors: A Comprehensive Literature Review with Emphasis on Their Clinical, Morphological and Genetic Features. Diagnostics, 15.","DOI":"10.3390\/diagnostics15070855"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"1554","DOI":"10.1097\/PAS.0b013e3181accc6c","article-title":"Hybrid Schwannoma\/Perineurioma: Clinicopathologic Analysis of 42 Distinctive Benign Nerve Sheath Tumors","volume":"33","author":"Hornick","year":"2009","journal-title":"Am. J. Surg. 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Dermatopathol."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"825","DOI":"10.4103\/0377-4929.91542","article-title":"Malignant Transformation in a Hybrid Schwannoma\/Perineurioma: Addition to the Spectrum of a Malignant Peripheral Nerve Sheath Tumor","volume":"54","author":"Rekhi","year":"2011","journal-title":"Indian J. Pathol. Microbiol."}],"container-title":["Cancers"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2072-6694\/18\/18\/3053\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,9,21]],"date-time":"2026-09-21T13:13:38Z","timestamp":1789996418000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2072-6694\/18\/18\/3053"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,9,20]]},"references-count":38,"journal-issue":{"issue":"18","published-online":{"date-parts":[[2026,9]]}},"alternative-id":["cancers18183053"],"URL":"https:\/\/doi.org\/10.3390\/cancers18183053","relation":{},"ISSN":["2072-6694"],"issn-type":[{"value":"2072-6694","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,9,20]]}}}