{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T05:04:52Z","timestamp":1790226292130,"version":"4.1.0"},"reference-count":74,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"3","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["FOC"],"published-print":{"date-parts":[[2013,3]]},"abstract":"<jats:sec>\n                    <jats:title>Object<\/jats:title>\n                    <jats:p>Malignant peripheral nerve sheath tumors (MPNSTs) are a rare form of malignancy arising from the Schwann cells of peripheral nerves. MPNSTs of the trigeminal nerve are exceptionally rare, with only a handful of reports in the literature. These tumors are typically very aggressive, resulting in significant patient morbidity and a generally grim prognosis. Most current reports suggest that radical resection with radiation therapy offers the best benefit. In this study, the authors systematically reviewed the world English-language literature on MPNSTs of the trigeminal nerve to analyze the presentations, treatment options, and outcomes for patients with this disease.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>A literature search for MPNSTs of the trigeminal nerve confined to nonanimal, English-language articles was conducted utilizing the PubMed database, with additional cases chosen from the references of selected articles. Only cases of confirmed MPNSTs of the trigeminal nerve or its peripheral branches, based upon surgical, pathological, or radiological analysis, were included.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>From the literature search, 29 articles discussing 35 cases of MPNSTs of the trigeminal nerve were chosen. With the addition of 1 case from their own institution, the authors analyzed 36 cases of trigeminal MPNSTs. The average age of onset was 44.6 years. These tumors were more commonly seen in male patients (77.1%). The gasserian ganglion was involved in 36.1% of the cases. Of the cases in which the nerve distribution was specified (n = 25), the mandibular branch was most commonly involved (72.0%), followed by the maxillary branch (60.0%) and the ophthalmic branch (32.0%), with 44.0% of patients exhibiting involvement of 2 or more branches. Altered facial sensation and facial pain were the 2 most commonly reported symptoms, found in 63.9% and 52.8% of patients, respectively. Mastication difficulty and diplopia were seen in 22.2% of patients, facial weakness was seen in 19.4%, and hearing loss was present in 16.7%. With regard to the primary treatment strategy, 80.6% underwent resection, 16.7% underwent radiation therapy, and 2.9% received chemotherapy alone. Patients treated with complete resection followed by postoperative radiation therapy had the most favorable outcomes, with no patients showing evidence of disease recurrence with a mean follow-up of 34.6 months. Patients treated with incomplete resection followed by postoperative radiation therapy had more favorable outcomes than patients treated with incomplete resection without radiation therapy or radiation therapy alone.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Trigeminal MPNSTs most commonly present as altered facial sensation or facial pain, although they exhibit a number of other clinical manifestations, including the involvement of other cranial nerves. While a variety of treatment options exist, due to their highly infiltrative nature, aggressive resection followed by radiation therapy appears to offer the greatest chance of recurrence-free survival.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3171\/2012.11.focus12292","type":"journal-article","created":{"date-parts":[[2013,3,1]],"date-time":"2013-03-01T16:13:48Z","timestamp":1362154428000},"page":"E5","source":"Crossref","is-referenced-by-count":29,"title":["Malignant peripheral nerve sheath tumors of the trigeminal nerve: a systematic review of 36 cases"],"prefix":"10.3171","volume":"34","author":[{"given":"Richard F.","family":"Schmidt","sequence":"first","affiliation":[{"name":"Departments of Neurological Surgery and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frederick","family":"Yick","sequence":"additional","affiliation":[{"name":"Departments of Neurological Surgery and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zain","family":"Boghani","sequence":"additional","affiliation":[{"name":"Departments of Neurological Surgery and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean Anderson","family":"Eloy","sequence":"additional","affiliation":[{"name":"Departments of Neurological Surgery and"},{"name":"Otolaryngology\u2013Head and Neck Surgery,"},{"name":"Center for Skull Base and Pituitary Surgery, Neurological Institute of New Jersey, University of Medicine and Dentistry of New Jersey\u2014New Jersey Medical School, Newark, New Jersey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James K.","family":"Liu","sequence":"additional","affiliation":[{"name":"Departments of Neurological Surgery and"},{"name":"Otolaryngology\u2013Head and Neck Surgery,"},{"name":"Center for Skull Base and Pituitary Surgery, Neurological Institute of New Jersey, University of Medicine and Dentistry of New Jersey\u2014New Jersey Medical School, Newark, New Jersey"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1754","reference":[{"key":"b1-0340005","series-title":"Acta Neurochir (Wien)","first-page":"591","article-title":"Primary intracranial malignant schwannoma of trigeminal nerve. A case report with review of the literature","volume":"142","author":"Akimoto","year":"2000"},{"key":"b2-0340005","series-title":"Laryngoscope","first-page":"1044","article-title":"Malignant nerve sheath tumors of the head and neck: a combined experience from two university hospitals","volume":"101","author":"Bailet","year":"1991"},{"key":"b3-0340005","series-title":"Neurosurgery","first-page":"500","article-title":"Malignant nerve sheath tumor of the right cerebral peduncle: case report","volume":"54","author":"Beauchesne","year":"2004"},{"key":"b4-0340005","series-title":"Br J Neurosurg","first-page":"750","article-title":"Malignant peripheral nerve sheath tumour of the trigeminal nerve: case report and literature review","volume":"25","author":"Bowers","year":"2011"},{"key":"b5-0340005","series-title":"Acta Neurochir (Wien)","first-page":"599","article-title":"Malignant trigeminal schwannoma extending into the anterior skull base","volume":"150","author":"Chibbaro","year":"2008"},{"key":"b6-0340005","series-title":"J Neurosurg","first-page":"423","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"},{"key":"b7-0340005","series-title":"Brain Pathol","first-page":"233","article-title":"Case of the month: June 1997\u2014a 42 year old man with left facial weakness","volume":"8","author":"DeRubeis","year":"1998"},{"key":"b8-0340005","series-title":"Neurol India","first-page":"43","article-title":"Malignant schwannoma of the trigeminal nerve","volume":"19","author":"Dinakar","year":"1971"},{"key":"b9-0340005","series-title":"Cancer","first-page":"2006","article-title":"Malignant peripheral nerve sheath tumors. A clinicopathologic study of 120 cases","volume":"57","author":"Ducatman","year":"1986"},{"key":"b10-0340005","series-title":"J Neurooncol","first-page":"173","article-title":"Malignant epithelioid cranial nerve sheath tumor: case report of a radiation response","volume":"78","author":"Fisher","year":"2006"},{"key":"b11-0340005","series-title":"J Clin Neurosci","first-page":"485","article-title":"Clavicular metastasis from malignant trigeminal schwannoma","volume":"10","author":"Ganapathy","year":"2003"},{"key":"b12-0340005","series-title":"Neurosurg Clin N Am","first-page":"533","article-title":"Malignant peripheral nerve sheath tumors","volume":"19","author":"Gupta","year":"2008"},{"key":"b13-0340005","series-title":"Cancer","first-page":"3549","article-title":"Peripheral nerve sheath tumors. Histogenesis, classification, and prognosis","volume":"72","author":"Hajdu","year":"1993"},{"key":"b14-0340005","series-title":"J Neurosurg","first-page":"279","article-title":"Primary malignant schwannoma of the Gasserian ganglion. Report of two cases","volume":"48","author":"Hedeman","year":"1978"},{"key":"b15-0340005","series-title":"Clin Neuropathol","first-page":"115","article-title":"Malignant peripheral nerve sheath tumors developing multifocally in the central nervous system in a patient with neurofibromatosis type 2","volume":"17","author":"Higami","year":"1998"},{"key":"b16-0340005","series-title":"Acta Pathol Jpn","first-page":"219","article-title":"Malignant schwannoma arising in the intracranial trigeminal nerve. A report of an autopsy case and a review of the literature","volume":"40","author":"Horie","year":"1990"},{"key":"b17-0340005","series-title":"Otolaryngol Head Neck Surg (1979)","first-page":"594","article-title":"Malignant schwannoma of the trigeminal nerve","volume":"87","author":"Karmody","year":"1979"},{"key":"b18-0340005","series-title":"Sao Paulo Med J","first-page":"333","article-title":"Description of a neural sheath tumor of the trigeminal nerve: immunohistochemical and electron microscopy study","volume":"124","author":"Khademi","year":"2006"},{"key":"b19-0340005","series-title":"AJNR Am J Neuroradiol","first-page":"748","article-title":"Malignant peripheral nerve sheath tumor in the parapharyngeal space: tumor spread through the eustachian tube","volume":"22","author":"Lee","year":"2001"},{"key":"b20-0340005","series-title":"Neurosurgery","first-page":"572","article-title":"Primary malignant nerve sheath tumor of the gasserian ganglion: a report of two cases","volume":"13","author":"Levy","year":"1983"},{"key":"b21-0340005","series-title":"J Neurosurg","first-page":"253","article-title":"Bilateral trigeminal neurofibrosarcoma. Case report","volume":"50","author":"Liwnicz","year":"1979"},{"key":"b22-0340005","series-title":"Otolaryngol Head Neck Surg","first-page":"667","article-title":"Malignant peripheral nerve sheath tumors of the head and neck: analysis of prognostic factors","volume":"122","author":"Loree","year":"2000"},{"key":"b23-0340005","series-title":"Br J Neurosurg","first-page":"729","article-title":"Trigeminal schwannomas","volume":"22","author":"MacNally","year":"2008"},{"key":"b24-0340005","series-title":"Can J Neurol Sci","first-page":"146","article-title":"Primary malignant tumours of the trigeminal nerve","volume":"13","author":"Maroun","year":"1986"},{"key":"b25-0340005","series-title":"Head Neck","first-page":"439","article-title":"Malignant peripheral nerve sheath tumors of the head and neck: management of 10 cases and literature review","volume":"29","author":"Minovi","year":"2007"},{"key":"b26-0340005","series-title":"J Am Acad Dermatol","first-page":"349","article-title":"Malignant schwannoma associated with xeroderma pigmentosum in a patient belonging to complementation group D","volume":"25","author":"Nakamura","year":"1991"},{"key":"b27-0340005","series-title":"Int J Oral Maxillofac Surg","first-page":"280","article-title":"Extensive malignant schwannoma of the mandibular nerve. Case report","volume":"21","author":"Ohnishi","year":"1992"},{"key":"b28-0340005","series-title":"Pathology","first-page":"421","article-title":"Malignant schwannoma of cranial nerves","volume":"15","author":"Robertson","year":"1983"},{"key":"b29-0340005","series-title":"J Neuroophthalmol","first-page":"29","article-title":"Painful sixth cranial nerve palsy caused by a malignant trigeminal nerve sheath tumor","volume":"27","author":"Rodr\u00edguez","year":"2007"},{"key":"b30-0340005","series-title":"AJNR Am J Neuroradiol","first-page":"396","article-title":"Perineural invasion through the maxillary division of the right trigeminal nerve in a rare case of nasolabial malignant peripheral nerve sheath tumor","volume":"29","author":"Sempere-Ortega","year":"2008"},{"key":"b31-0340005","series-title":"Acta Neurochir (Wien)","first-page":"357","article-title":"Malignant peripheral nerve sheath tumours\u2014report of 8 cases and review of the literature","volume":"143","author":"Stark","year":"2001"},{"key":"b32-0340005","series-title":"Neurosurgery","first-page":"E425","article-title":"Chronic recurrent subarachnoid hemorrhage from a trigeminal nerve malignant peripheral nerve sheath tumor: case report","volume":"59","author":"Stark","year":"2006"},{"key":"b33-0340005","series-title":"AJNR Am J Neuroradiol","first-page":"505","article-title":"Malignant schwannoma of the trigeminal nerve","volume":"22","author":"Stone","year":"2001"},{"key":"b34-0340005","series-title":"J Neurosurg Sci","first-page":"293","article-title":"Primary malignant schwannoma involving simultaneously the right Gasserian ganglion and the distal part of the right mandibular nerve. Case report","volume":"41","author":"Tegos","year":"1997"},{"key":"b35-0340005","series-title":"Neurol Med Chir (Tokyo)","first-page":"38","article-title":"Malignant peripheral nerve sheath tumor in the anterior skull base associated with neurofibromatosis type 1\u2014case report","volume":"44","author":"Ueda","year":"2004"},{"key":"b36-0340005","series-title":"Neurol Med Chir (Tokyo)","first-page":"817","article-title":"Malignant trigeminal schwannoma associated with xeroderma pigmentosum\u2014case report","volume":"34","author":"Yamashiro","year":"1994"},{"key":"b37-0340005","series-title":"Auris Nasus Larynx","first-page":"539","article-title":"Malignant peripheral nerve sheath tumor of intracranial nerve: a case series review","volume":"37","author":"Ziadi","year":"2010"},{"key":"b1-0340005","series-title":"Acta Neurochir (Wien)","first-page":"591","article-title":"Primary intracranial malignant schwannoma of trigeminal nerve. A case report with review of the literature","volume":"142","author":"Akimoto","year":"2000"},{"key":"b2-0340005","series-title":"Laryngoscope","first-page":"1044","article-title":"Malignant nerve sheath tumors of the head and neck: a combined experience from two university hospitals","volume":"101","author":"Bailet","year":"1991"},{"key":"b3-0340005","series-title":"Neurosurgery","first-page":"500","article-title":"Malignant nerve sheath tumor of the right cerebral peduncle: case report","volume":"54","author":"Beauchesne","year":"2004"},{"key":"b4-0340005","series-title":"Br J Neurosurg","first-page":"750","article-title":"Malignant peripheral nerve sheath tumour of the trigeminal nerve: case report and literature review","volume":"25","author":"Bowers","year":"2011"},{"key":"b5-0340005","series-title":"Acta Neurochir (Wien)","first-page":"599","article-title":"Malignant trigeminal schwannoma extending into the anterior skull base","volume":"150","author":"Chibbaro","year":"2008"},{"key":"b6-0340005","series-title":"J Neurosurg","first-page":"423","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"},{"key":"b7-0340005","series-title":"Brain Pathol","first-page":"233","article-title":"Case of the month: June 1997\u2014a 42 year old man with left facial weakness","volume":"8","author":"DeRubeis","year":"1998"},{"key":"b8-0340005","series-title":"Neurol India","first-page":"43","article-title":"Malignant schwannoma of the trigeminal nerve","volume":"19","author":"Dinakar","year":"1971"},{"key":"b9-0340005","series-title":"Cancer","first-page":"2006","article-title":"Malignant peripheral nerve sheath tumors. A clinicopathologic study of 120 cases","volume":"57","author":"Ducatman","year":"1986"},{"key":"b10-0340005","series-title":"J Neurooncol","first-page":"173","article-title":"Malignant epithelioid cranial nerve sheath tumor: case report of a radiation response","volume":"78","author":"Fisher","year":"2006"},{"key":"b11-0340005","series-title":"J Clin Neurosci","first-page":"485","article-title":"Clavicular metastasis from malignant trigeminal schwannoma","volume":"10","author":"Ganapathy","year":"2003"},{"key":"b12-0340005","series-title":"Neurosurg Clin N Am","first-page":"533","article-title":"Malignant peripheral nerve sheath tumors","volume":"19","author":"Gupta","year":"2008"},{"key":"b13-0340005","series-title":"Cancer","first-page":"3549","article-title":"Peripheral nerve sheath tumors. Histogenesis, classification, and prognosis","volume":"72","author":"Hajdu","year":"1993"},{"key":"b14-0340005","series-title":"J Neurosurg","first-page":"279","article-title":"Primary malignant schwannoma of the Gasserian ganglion. Report of two cases","volume":"48","author":"Hedeman","year":"1978"},{"key":"b15-0340005","series-title":"Clin Neuropathol","first-page":"115","article-title":"Malignant peripheral nerve sheath tumors developing multifocally in the central nervous system in a patient with neurofibromatosis type 2","volume":"17","author":"Higami","year":"1998"},{"key":"b16-0340005","series-title":"Acta Pathol Jpn","first-page":"219","article-title":"Malignant schwannoma arising in the intracranial trigeminal nerve. A report of an autopsy case and a review of the literature","volume":"40","author":"Horie","year":"1990"},{"key":"b17-0340005","series-title":"Otolaryngol Head Neck Surg (1979)","first-page":"594","article-title":"Malignant schwannoma of the trigeminal nerve","volume":"87","author":"Karmody","year":"1979"},{"key":"b18-0340005","series-title":"Sao Paulo Med J","first-page":"333","article-title":"Description of a neural sheath tumor of the trigeminal nerve: immunohistochemical and electron microscopy study","volume":"124","author":"Khademi","year":"2006"},{"key":"b19-0340005","series-title":"AJNR Am J Neuroradiol","first-page":"748","article-title":"Malignant peripheral nerve sheath tumor in the parapharyngeal space: tumor spread through the eustachian tube","volume":"22","author":"Lee","year":"2001"},{"key":"b20-0340005","series-title":"Neurosurgery","first-page":"572","article-title":"Primary malignant nerve sheath tumor of the gasserian ganglion: a report of two cases","volume":"13","author":"Levy","year":"1983"},{"key":"b21-0340005","series-title":"J Neurosurg","first-page":"253","article-title":"Bilateral trigeminal neurofibrosarcoma. Case report","volume":"50","author":"Liwnicz","year":"1979"},{"key":"b22-0340005","series-title":"Otolaryngol Head Neck Surg","first-page":"667","article-title":"Malignant peripheral nerve sheath tumors of the head and neck: analysis of prognostic factors","volume":"122","author":"Loree","year":"2000"},{"key":"b23-0340005","series-title":"Br J Neurosurg","first-page":"729","article-title":"Trigeminal schwannomas","volume":"22","author":"MacNally","year":"2008"},{"key":"b24-0340005","series-title":"Can J Neurol Sci","first-page":"146","article-title":"Primary malignant tumours of the trigeminal nerve","volume":"13","author":"Maroun","year":"1986"},{"key":"b25-0340005","series-title":"Head Neck","first-page":"439","article-title":"Malignant peripheral nerve sheath tumors of the head and neck: management of 10 cases and literature review","volume":"29","author":"Minovi","year":"2007"},{"key":"b26-0340005","series-title":"J Am Acad Dermatol","first-page":"349","article-title":"Malignant schwannoma associated with xeroderma pigmentosum in a patient belonging to complementation group D","volume":"25","author":"Nakamura","year":"1991"},{"key":"b27-0340005","series-title":"Int J Oral Maxillofac Surg","first-page":"280","article-title":"Extensive malignant schwannoma of the mandibular nerve. Case report","volume":"21","author":"Ohnishi","year":"1992"},{"key":"b28-0340005","series-title":"Pathology","first-page":"421","article-title":"Malignant schwannoma of cranial nerves","volume":"15","author":"Robertson","year":"1983"},{"key":"b29-0340005","series-title":"J Neuroophthalmol","first-page":"29","article-title":"Painful sixth cranial nerve palsy caused by a malignant trigeminal nerve sheath tumor","volume":"27","author":"Rodr\u00edguez","year":"2007"},{"key":"b30-0340005","series-title":"AJNR Am J Neuroradiol","first-page":"396","article-title":"Perineural invasion through the maxillary division of the right trigeminal nerve in a rare case of nasolabial malignant peripheral nerve sheath tumor","volume":"29","author":"Sempere-Ortega","year":"2008"},{"key":"b31-0340005","series-title":"Acta Neurochir (Wien)","first-page":"357","article-title":"Malignant peripheral nerve sheath tumours\u2014report of 8 cases and review of the literature","volume":"143","author":"Stark","year":"2001"},{"key":"b32-0340005","series-title":"Neurosurgery","first-page":"E425","article-title":"Chronic recurrent subarachnoid hemorrhage from a trigeminal nerve malignant peripheral nerve sheath tumor: case report","volume":"59","author":"Stark","year":"2006"},{"key":"b33-0340005","series-title":"AJNR Am J Neuroradiol","first-page":"505","article-title":"Malignant schwannoma of the trigeminal nerve","volume":"22","author":"Stone","year":"2001"},{"key":"b34-0340005","series-title":"J Neurosurg Sci","first-page":"293","article-title":"Primary malignant schwannoma involving simultaneously the right Gasserian ganglion and the distal part of the right mandibular nerve. 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