ArticleOncoTargets and therapy2013
Updates on the diagnosis and treatment of intracranial nerve malignant peripheral nerve sheath tumors.
Article in OncoTargets and therapy, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.
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Who cites it
17 citing papers in PubMed, 39 citations in OpenAlex.
- Clinical and genomic profiles of malignant peripheral nerve sheath tumors in the craniospinal axis.Journal of neuro-oncology · 2025Article
- Malignant Transformation of a Vestibular Schwannoma Without Previous Radiation Exposure: Illustrative Case and Literature Review.Journal of neurological surgery reports · 2025Article
- Clinical Findings and Outcome in 30 Dogs with Presumptive or Confirmed Nerve Sheath Tumors.Veterinary sciences · 2024Article
- A Rare Case of Malignant Peripheral Nerve Sheath Tumor of Larynx.Indian journal of otolaryngology and head and neck surgery : official publication of the Association of Otolaryngologists of India · 2022Article
- Curious Case of Acoustic Schwannoma of Middle Cranial Fossa: A Case Report.Brain tumor research and treatment · 2022Article
- Cranial and Spinal Malignant Peripheral Nerve Sheath Tumor: A Pathological Enigma.Journal of neurosciences in rural practice · 2021Article
- Parotid Neurogenic Tumors: MPNST Sarcoma to Schwannoma-Review of Literature and Guidelines in Management.Journal of maxillofacial and oral surgery · 2021Review
- First documented case of intracranial falcine malignant peripheral nerve sheath tumor: illustrative case.Journal of neurosurgery. Case lessons · 2021Article
- Malignant intracerebral nerve sheath tumor in a patient with Noonan syndrome: illustrative case.Journal of neurosurgery. Case lessons · 2021Article
- Article
- Not So Benign Bell's Palsy: Malignant Peripheral Nerve Sheath Tumor of the Facial Nerve Involving the Temporal Bone.Journal of general internal medicine · 2021Article
- Incidence and prognosis of distant metastasis in malignant peripheral nerve sheath tumors.Acta neurochirurgica · 2021Article
- Treatment and survival differences across tumor sites in malignant peripheral nerve sheath tumors: a SEER database analysis and review of the literature.Neuro-oncology practice · 2019Article
- Surgical treatment of rare giant malignant tumors of the scalp: A report of 3 cases with different tumor types.Oncology letters · 2016Article
- Cancer around the brain.Neuro-oncology practice · 2014Review
- Malignant peripheral nerve sheath tumors of the head and neck: a case series and literature review.Case reports in otolaryngology · 2014Article
- Intracranial Malignant Nerve Sheath Tumor in the Middle Cranial Fossa: A Rare Case Report with Review of Literature.Asian journal of neurosurgeryArticle
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Authors and funding
2 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundMalignant peripheral nerve sheath tumors (MPNSTs) are rare entities and MPNSTs of intracranial nerves are even more sporadic. MPNSTs present diagnosis and treatment challenges since there are no defined diagnosis criteria and no established therapeutic strategies.
methodsWe reviewed literature for MPNST-related articles. We found 45 relevant studies in which 60 cases were described.
resultsWe identified 60 cases of intracranial nerve MPNSTs. The age ranged from 3 to 75 years old. Male to female ratio was 1.5:1. The most involved cranial nerves (CNs) were CN VIII (60%), CN V (27%), and CN VII (10%). Most of the MPNSTs reported (47%) arose sporadically, 40% arose from a schwannoma, 8% arose from a neurofibroma, and 6% arose from an unspecified nerve tumor. Twenty patients had a history of radiation exposure, four patients had neurofibromatosis type 1 (NF1), four patients had neurofibromatosis type 2 (NF2), and NF2 was suspected in two other patients. Twenty-two patients were treated with radiotherapy and presented a higher survival rate. Seventy-two percent of patients died of their disease while 28% of patients survived. One-year survival rate was 33%. Forty-five percent of tumors recurred and 19% of patients had metastases.
conclusionMPNSTs involving CNs are very rare. Diagnosis is made in regards to the histological and pathological findings. Imaging may help orient the diagnosis. A preexisting knowledge of the clinical situation is more likely to lead to a correct diagnosis. The mainstay of treatment is radical surgical resection with adjuvant radiotherapy. Since these tumors are associated with a poor prognosis, a close follow-up is mandatory.
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