{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T04:33:12Z","timestamp":1791261192816,"version":"4.1.0"},"reference-count":68,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"3","license":[{"start":{"date-parts":[[2021,2,16]],"date-time":"2021-02-16T00:00:00Z","timestamp":1613433600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Neurosurg.","NEUROS"],"published-print":{"date-parts":[[2021,3]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Neoplasms of the peripheral nervous system represent a heterogenous group with a wide spectrum of morphological features and biological potential. They range from benign and curable by complete excision (schwannoma and soft tissue perineurioma) to benign but potentially aggressive at the local level (plexiform neurofibroma) to the highly malignant (malignant peripheral nerve sheath tumors [MPNST]). In this review, we discuss the diagnostic and pathologic features of common peripheral nerve sheath tumors, particularly those that may be encountered in the intracranial compartment or in the spine and paraspinal region. The discussion will cover schwannoma, neurofibroma, atypical neurofibromatous neoplasms of uncertain biological potential, intraneural and soft tissue perineurioma, hybrid nerve sheath tumors, MPNST, and the recently renamed enigmatic tumor, malignant melanotic nerve sheath tumor, formerly referred to as melanotic schwannoma. We also discuss the diagnostic relevance of these neoplasms to specific genetic and familial syndromes of nerve, including neurofibromatosis 1, neurofibromatosis 2, and schwannomatosis. In addition, we discuss updates in our understanding of the molecular alterations that represent key drivers of these neoplasms, including neurofibromatosis type 1 and type 2,\n                    <jats:italic toggle=\"yes\">SMARCB1, LZTR1<\/jats:italic>\n                    , and\n                    <jats:italic toggle=\"yes\">PRKAR1A<\/jats:italic>\n                    loss, as well as the acquisition of\n                    <jats:italic toggle=\"yes\">CDKN2A\/B<\/jats:italic>\n                    mutations and alterations in the polycomb repressor complex members (\n                    <jats:italic toggle=\"yes\">SUZ12<\/jats:italic>\n                    and\n                    <jats:italic toggle=\"yes\">EED<\/jats:italic>\n                    ) in the malignant progression to MPNST. In summary, this review covers practical aspects of pathologic diagnosis with updates relevant to neurosurgical practice.\n                  <\/jats:p>","DOI":"10.1093\/neuros\/nyab021","type":"journal-article","created":{"date-parts":[[2021,2,2]],"date-time":"2021-02-02T23:43:34Z","timestamp":1612309414000},"page":"443-456","source":"Crossref","is-referenced-by-count":146,"title":["Diagnostic Pathology of Tumors of Peripheral Nerve"],"prefix":"10.1227","volume":"88","author":[{"given":"Sarra M","family":"Belakhoua","sequence":"first","affiliation":[{"name":"Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA"},{"name":"School of Medicine, University of Tunis El Manar, Tunis, Tunisia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8662-1219","authenticated-orcid":false,"given":"Fausto J","family":"Rodriguez","sequence":"additional","affiliation":[{"name":"Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA"},{"name":"Department of Ophthalmology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA"},{"name":"Sydney Kimmel Cancer Center, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2021,3]]},"reference":[{"issue":"6","key":"bib1-20231011","doi-asserted-by":"crossref","first-page":"877","DOI":"10.1007\/s00401-016-1540-6","article-title":"Methylation-based classification of benign and malignant peripheral nerve sheath tumors","volume":"131","author":"Rohrich","year":"2016","journal-title":"Acta Neuropathol"},{"issue":"7","key":"bib2-20231011","doi-asserted-by":"crossref","first-page":"917","DOI":"10.1093\/neuonc\/noy009","article-title":"Targeted next-generation sequencing for differential diagnosis of neurofibromatosis type 2, schwannomatosis, and meningiomatosis","volume":"20","author":"Louvrier","year":"2018","journal-title":"Neuro Oncol"},{"issue":"9","key":"bib3-20231011","doi-asserted-by":"crossref","first-page":"e860","DOI":"10.1097\/MAO.0000000000001932","article-title":"Next generation sequencing of sporadic vestibular schwannoma: necessity of biallelic NF2 inactivation and implications of accessory non-NF2 variants","volume":"39","author":"Carlson","year":"2018","journal-title":"Otol Neurotol"},{"issue":"3","key":"bib4-20231011","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1007\/s00401-011-0935-7","article-title":"Genetic predisposition to peripheral nerve neoplasia: diagnostic criteria and pathogenesis of neurofibromatoses, Carney complex, and related syndromes","volume":"123","author":"Rodriguez","year":"2012","journal-title":"Acta Neuropathol"},{"issue":"1","key":"bib5-20231011","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1212\/WNL.0000000000003418","article-title":"Revisiting neurofibromatosis type 2 diagnostic criteria to exclude LZTR1-related schwannomatosis","volume":"88","author":"Smith","year":"2017","journal-title":"Neurology"},{"issue":"2","key":"bib6-20231011","doi-asserted-by":"crossref","first-page":"217","DOI":"10.3171\/jns.1993.79.2.0217","article-title":"Intracranial parenchymal schwannoma. 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