{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T15:28:50Z","timestamp":1791559730003,"version":"4.3.4"},"reference-count":104,"publisher":"MDPI AG","issue":"2","license":[{"start":{"date-parts":[[2025,1,8]],"date-time":"2025-01-08T00:00:00Z","timestamp":1736294400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"NINDS","award":["R01NS134815-02"],"award-info":[{"award-number":["R01NS134815-02"]}]},{"name":"St. Louis Men\u2019s Group Against Cancer","award":["R01NS134815-02"],"award-info":[{"award-number":["R01NS134815-02"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cancers"],"abstract":"<jats:p>Malignant peripheral nerve sheath tumors (MPNSTs) are rare but aggressive malignancies with a low 5-year survival rate despite current treatments. MPNSTs frequently harbor mutations in key genes such as NF1, CDKN2A, TP53, and PRC2 components (EED or SUZ12) across different disease stages. With the rapid advancement of high-throughput sequencing technologies, the molecular characteristics driving MPNST development are becoming clearer. This review summarizes recent sequencing studies on peripheral nerve sheath tumors, including plexiform neurofibromas (PNs), atypical neurofibromatous neoplasm with uncertain biologic potential (ANNUBP), and MPNSTs, highlighting key mutation events in tumor progression from the perspectives of epigenetics, transcriptomics, genomics, proteomics, and metabolomics. We also discuss the therapeutic implications of these genomic findings, focusing on preclinical and clinical trials targeting these alterations. Finally, we conclude that overcoming tumor resistance through combined targeted therapies and personalized treatments based on the molecular characteristics of MPNSTs will be a key direction for future treatment strategies.<\/jats:p>","DOI":"10.3390\/cancers17020180","type":"journal-article","created":{"date-parts":[[2025,1,8]],"date-time":"2025-01-08T04:54:08Z","timestamp":1736312048000},"page":"180","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["A Sequencing Overview of Malignant Peripheral Nerve Sheath Tumors: Findings and Implications for Treatment"],"prefix":"10.3390","volume":"17","author":[{"given":"Kangwen","family":"Xiao","sequence":"first","affiliation":[{"name":"Division of Oncology, Department of Internal Medicine, Siteman Cancer Center, Washington University School of Medicine, St. Louis, MO 63110, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6992-9585","authenticated-orcid":false,"given":"Kuangying","family":"Yang","sequence":"additional","affiliation":[{"name":"Division of Oncology, Department of Internal Medicine, Siteman Cancer Center, Washington University School of Medicine, St. Louis, MO 63110, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1719-0771","authenticated-orcid":false,"given":"Angela C.","family":"Hirbe","sequence":"additional","affiliation":[{"name":"Division of Oncology, Department of Internal Medicine, Siteman Cancer Center, Washington University School of Medicine, St. Louis, MO 63110, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,1,8]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"423","DOI":"10.3171\/jns.1952.9.5.0423","article-title":"Tumors of the Gasserian Ganglion: Tumor of the Left Gasserian Ganglion Associated with Enlargement of the Mandibular Nerve a Review of the Literature and Case Report","volume":"9","author":"Cuneo","year":"1952","journal-title":"J. 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