{"id":"71beb42d-4b0f-4acc-bb22-592dc8124cf1","slug":"intracranial-malignant-peripheral-nerve-sheath-tumor-a-case-report-and-comprehensive-literature-review","title":"Intracranial malignant peripheral nerve sheath tumor: A case report and comprehensive literature review","authors":["Brandon Michael Wilkinson","Michael A. Duncan","Richard Davila","Brian Nicholas","Harish Babu"],"abstract":"Background: Malignant peripheral nerve sheath tumors (MPNSTs) are rare malignant soft-tissue sarcomas arising from peripheral nerves. Little data exist regarding MPNST originating intracranially. Here, we present a 7th/8th nerve complex MPNST, discuss the treatment strategy and patient outcome, and provide a comprehensive review of existing literature. Methods: Using Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, PubMed and crossed references were queried, yielding 37 publications from 1952 to the present. Fifty-three cases of primary intracranial and extra-axial MPNST were identified. Results: We additionally report a 40-year-old female presented with acute onset dizziness and subsequent hearing loss with associated right-sided facial numbness. Magnetic resonance imaging revealed a 0.5 cm × 1.7 cm enhancing lesion within the right internal auditory canal extending into the cerebellopontine angle. The patient was initially treated with retro sigmoid craniotomy for tumor resection followed by a trans labyrinth approach for residual tumor resection. She completed adjuvant fractionated radiation therapy and underwent facial nerve transfer to restore complete hemifacial paralysis. The most common cranial nerves involved were V and VIII (43.4% each), with 66% of patients male and 34% female. The average age was 43.4 ± 17.4 years. The mean survival time for reported non-survivors after tissue diagnosis was 15 ± 4 months. Two-year survival for patients receiving gross total resection was 33.3% versus 22.8% with subtotal resection. Conclusion: MPNSTs comprise a group of highly aggressive neoplasms that rarely arise intracranially. Gross total surgical resection should be pursued when feasible.","thumbnailUrl":"https://sni-digital-videos.s3.amazonaws.com/articles/71beb42d-4b0f-4acc-bb22-592dc8124cf1/featured/hero-1781559659530.png","publishDate":"2024-03-22T00:00:00.000Z","doi":"10.25259/SNI_931_2023","categories":["Skull Base","Review Article"],"fullTextUrl":"https://surgicalneurologyint.com/wp-content/uploads/2024/03/12812/SNI-15-101.pdf"}