{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T19:11:19Z","timestamp":1791313879205,"version":"4.3.1"},"reference-count":93,"publisher":"MDPI AG","issue":"6","license":[{"start":{"date-parts":[[2022,6,14]],"date-time":"2022-06-14T00:00:00Z","timestamp":1655164800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Diagnostics"],"abstract":"<jats:p>Peripheral nerve sheath tumors encompass a wide spectrum of lesions with different biological behavior, including both benign and malignant neoplasms as well as the recent diagnostic category, i.e., \u201catypical neurofibromatous neoplasm with uncertain biologic potential\u201d to be used only for NF1 patients. Neurofibromas and schwannomas are benign Schwann-cell-derived peripheral nerve sheath tumors arising as isolated lesions or within the context of classical neurofibromatosis or schwannomatoses. Multiple tumors are a hallmark of neurofibromatosis type 1(NF1) and related forms, NF2-related-schwannomatosis (formerly NF2) or SMARCB1\/LZTR1-related schwannomatoses. Perineuriomas are benign, mostly sporadic, peripheral nerve sheath tumors that show morphological, immunohistochemical, and ultrastructural features reminiscent of perineurial differentiation. Hybrid tumors exist, with the most common lesions represented by a variable mixture of neurofibromas, schwannomas, and perineuriomas. Conversely, malignant peripheral nerve sheath tumors are soft tissue sarcomas that may arise from a peripheral nerve or a pre-existing neurofibroma, and in about 50% of cases, these tumors are associated with NF1. The present review emphasizes the main clinicopathologic features of each pathological entity, focusing on the diagnostic clues and unusual morphological variants.<\/jats:p>","DOI":"10.3390\/diagnostics12061463","type":"journal-article","created":{"date-parts":[[2022,6,14]],"date-time":"2022-06-14T23:50:14Z","timestamp":1655250614000},"page":"1463","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":100,"title":["Practical Approach to Histological Diagnosis of Peripheral Nerve Sheath Tumors: An Update"],"prefix":"10.3390","volume":"12","author":[{"given":"Gaetano","family":"Magro","sequence":"first","affiliation":[{"name":"Anatomic Pathology, Department of Medical, Surgical Sciences and Advanced Technologies \u201cG.F. Ingrassia\u201d, University of Catania, 95123 Catania, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2576-6523","authenticated-orcid":false,"given":"Giuseppe","family":"Broggi","sequence":"additional","affiliation":[{"name":"Anatomic Pathology, Department of Medical, Surgical Sciences and Advanced Technologies \u201cG.F. Ingrassia\u201d, University of Catania, 95123 Catania, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4808-2052","authenticated-orcid":false,"given":"Giuseppe","family":"Angelico","sequence":"additional","affiliation":[{"name":"Pathology Unit, Cannizzaro Hospital, 95126 Catania, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0466-1389","authenticated-orcid":false,"given":"Lidia","family":"Puzzo","sequence":"additional","affiliation":[{"name":"Anatomic Pathology, Department of Medical, Surgical Sciences and Advanced Technologies \u201cG.F. Ingrassia\u201d, University of Catania, 95123 Catania, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6377-9678","authenticated-orcid":false,"given":"Giada Maria","family":"Vecchio","sequence":"additional","affiliation":[{"name":"Anatomic Pathology, Department of Medical, Surgical Sciences and Advanced Technologies \u201cG.F. Ingrassia\u201d, University of Catania, 95123 Catania, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valentina","family":"Virz\u00ec","sequence":"additional","affiliation":[{"name":"Unit of Diagnostic Radiology, Regina Pacis Clinic, 93017 San Cataldo, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lucia","family":"Salvatorelli","sequence":"additional","affiliation":[{"name":"Anatomic Pathology, Department of Medical, Surgical Sciences and Advanced Technologies \u201cG.F. Ingrassia\u201d, University of Catania, 95123 Catania, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martino","family":"Ruggieri","sequence":"additional","affiliation":[{"name":"Unit of Rare Diseases of the Nervous System in Childhood, Department of Clinical and Experimental Medicine, University of Catania, 95124 Catania, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,6,14]]},"reference":[{"key":"ref_1","unstructured":"Goldblum, J.R., Folpe, A.L., and Weiss, S.W. (2020). Enzinger & Weiss\u2019s Soft Tissue Tumors, Elsevier. [7th ed.]."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1506","DOI":"10.1038\/s41436-021-01170-5","article-title":"Revised diagnostic criteria for neurofibromatosis type 1 and Legius syndrome: An international consensus recommendation","volume":"23","author":"Legius","year":"2021","journal-title":"Genet. 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