title: Malignant peripheral nerve sheath tumors of the trigeminal nerve: a systematic review of 36 cases.
abstract: OBJECTIVE
  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.
  
  
  METHODS
  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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors: Department of Neurological Surgery, Neurological Institute of New Jersey, University of Medicine and Dentistry of New Jersey—New Jersey Medical School, Newark, New Jersey, USA.
authors: Richard F
authors: RF
authors: Schmidt
authors: Frederick
authors: F
authors: Yick
authors: Zain
authors: Z
authors: Boghani
authors: Jean Anderson
authors: JA
authors: Eloy
authors: James K
authors: JK
authors: Liu
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D003131:Combined Modality Therapy; D003390:Cranial Nerve Neoplasms / Q000188:drug therapy / Q000453:epidemiology / Q000473:pathology / Q000532:radiotherapy / Q000601:surgery*; D004172:Diplopia / Q000209:etiology; D005156:Facial Neuralgia / Q000209:etiology; D005260:Female; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local; D018317:Nerve Sheath Neoplasms / Q000188:drug therapy / Q000453:epidemiology / Q000473:pathology / Q000532:radiotherapy / Q000601:surgery*; D009493:Neurosurgery / Q000379:methods; D011379:Prognosis; D018714:Radiotherapy, Adjuvant; D016896:Treatment Outcome; D020433:Trigeminal Nerve Diseases / Q000188:drug therapy / Q000453:epidemiology / Q000473:pathology / Q000532:radiotherapy / Q000601:surgery*; D055815:Young Adult