2021
DOI: 10.3171/case21146
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Malignant intracerebral nerve sheath tumor in a patient with Noonan syndrome: illustrative case

Abstract: BACKGROUND Malignant peripheral nerve sheath tumors (MPNSTs) within the neuroaxis are rare, usually arising from peripheral and cranial nerves. Even more scarce are cranial subclassifications of MPNSTs termed “malignant intracerebral nerve sheath tumors” (MINSTs). These tumors are aggressive, with a strong tendency for metastasis. With this presentation, alongside resistance to adjunctive therapy, complete excision is the mainstay of treatment, although it is often insufficient, resulting in a high rate of mor… Show more

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Cited by 2 publications
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“…It was finally found to be IDH1-R132 and IDH2-R172 wild type on Sanger sequencing, with the absence of mutations in histone H3F3Agene or telomerase reverse transcriptase (TERT) promoter genes. 7 MINST are aggressive tumours with varying outcomes with many having a dismal prognosis 8 Sharma et al studied that the earlier the first recurrence, the worse the overall survival of the patient. Charles et al did a systematic review of 56 patients with intracranial MPNSTs from 743 literature results.…”
Section: Discussionmentioning
confidence: 99%
“…It was finally found to be IDH1-R132 and IDH2-R172 wild type on Sanger sequencing, with the absence of mutations in histone H3F3Agene or telomerase reverse transcriptase (TERT) promoter genes. 7 MINST are aggressive tumours with varying outcomes with many having a dismal prognosis 8 Sharma et al studied that the earlier the first recurrence, the worse the overall survival of the patient. Charles et al did a systematic review of 56 patients with intracranial MPNSTs from 743 literature results.…”
Section: Discussionmentioning
confidence: 99%