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Malignant Peripheral Nerve Sheath Tumors of the Trigeminal Nerve: a Systematic Review of 36 Cases

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Malignant Peripheral Nerve Sheath Tumors of the Trigeminal Nerve: a Systematic Review of 36 Cases Neurosurg Focus 34 (3):E5, 2013 ©AANS, 2013 Malignant peripheral nerve sheath tumors of the trigeminal nerve: a systematic review of 36 cases RICHARD F. SCHMIDT, B.A.,1 FREDERICK YICK, B.A.,1 ZAIN BOGHANI, B.S.,1 JEAN ANDErsON ELOY, M.D.,1–3 AND JAMES K. LIU, M.D. 1–3 Departments of 1Neurological Surgery and 2Otolaryngology–Head and Neck Surgery, 3Center for Skull Base and Pituitary Surgery, Neurological Institute of New Jersey, University of Medicine and Dentistry of New Jersey—New Jersey Medical School, Newark, New Jersey Object. 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. (http://thejns.org/doi/abs/10.3171/2012.11.FOCUS12292) KEY WORDS • trigeminal nerve • malignancy • nerve sheath • peripheral nerve • cranial nerve • schwannoma • systematic analysis ALIGNANT peripheral nerve sheath tumors (al­ reported to have an increased risk of developing MPNSTs so known as neurofibrosarcomas or malignant in their lifetime.9 However, this association is not seen in schwan nomas) are extremely rare sarcomas MPNSTs of the cranial nerves.1,33 MPNSTs are typically Marising from the Schwann cells of peripheral nerves.5,12 found in the trunk and extremities and are not commonly These tumors have an incidence of approximately 0.001% seen in the head and neck region.31 MPNSTs of the cra­ in the general population13 and tend to present in older nial nerves are an exceptionally rare manifestation of this patients.12 Individuals with a family history of NF1 are disease, as most schwannomas of the cranial nerves are benign.1,3,21,33 MPNSTs of the trigeminal nerve are exceed­ Abbreviations used in this paper: MPNST = malignant peripheral ingly rare with very few existing reports in the literature. nerve sheath tumor; NF = neurofibromatosis; NF1 = NF Type 1; In this study, we performed a systematic review of 36 cas­ NF2 = NF Type 2. es of MPNSTs of the trigeminal nerve and analyzed the Neurosurg Focus / Volume 34 / March 2013 1 Unauthenticated | Downloaded 09/24/21 07:19 AM UTC R. F. Schmidt et al. clinical presentation, tumor distribution, treatment strate­ sion of the trigeminal nerve [V3]). Clinical presentation gies, and outcomes for patients with this disease. or distribution of symptoms (for example, pain or sensory disturbance in a specific distribution) was not used as a criterion for determining location, as we felt that this may Methods not accurately depict exactly where the tumor occurred. Search Strategy “Not available” was also assigned to certain cases if in­ volvement of the gasserian ganglion was specified but not A literature search for MPNSTs of the trigeminal the other nerve distributions. Tumors affecting peripheral nerve was conducted utilizing the PubMed database. The and terminal branches of the trigeminal nerve were in­ search was confined to nonanimal, English-language ar­ cluded when involvement of these nerves was specified. ticles. The following search terms were used: “trigeminal Clinical presentation was determined on the basis of the AND sheath tumor,” “trigeminal AND malignant periph­ reported signs or symptoms. eral nerve sheath tumor,” “trigeminal AND malignant,” “trigeminal AND nerve sheath tumor,” “trigeminal AND Data Analysis neural sheath tumor,” “trigeminal AND malignant tu­ mor,” “trigeminal AND malignant schwannoma,” “tri­ Microsoft Excel was used for data aggregation and geminal AND neurofibrosarcoma,” and “trigeminal analysis. AND neurosarcoma.” The references of included articles were searched for any additional pertinent studies. Results Inclusion Criteria Searching the PubMed database using the key words listed above identified 175 studies (Fig. 1). Exclusion cri­ We included all English-language articles—case re­ teria included other tumor (85 articles), treatments/tech­ ports, case illustrations, and case series. Relevant articles niques not pertaining to MPNSTs (27), radiology (10), were determined by identifying reports of patients with other disease (9), natural history (7), pathology (4), bio­ confirmed MPNSTs of the trigeminal nerve. Patients chemistry (3), pharmacology (3), anatomy (2), and anes­ were included in our study if specific trigeminal involve­ thesia (1). After applying the aforementioned criteria, 24 ment was reported, either by specific declaration of tri­ articles were included in the systematic review. A search geminal nerve involvement or description of anatomical of the references of the 24 articles yielded 5 additional distributions specific to trigeminal nerve distributions pertinent articles. These 29 articles provided 35 cases, (for example, foramen ovale). Papers discussing tumors to which we added 1 additional case from our institu­ based on location with an unspecified nerve origin or tion.1,2,4–8,10,11,14–21,24,26–30,32–37 The complete results of our nonspecific anatomical structures (for example, maxil­ analysis of 36 cases are detailed in Table 1. lary sinus MPNST) were not included in the study. One patient was reported as having bilateral MPNSTs that Patient Demographics were diagnosed and treated separately during 2 sepa­ rate time periods with different treatment regimens. As The patients’ ages ranged from 8 to 71 years (mean such, these lesions have been incorporated into our re­ 44.6 years). Eight patients were female (22.9%) and 27 pa­ tients were male (77.1%). Neurofibromatosis Type 1 was view as 2 separate cases, except in the analyses of sex present in 1 patient (2.9%), absent in 20 patients (57.1%), and NF status. The selected reports were reviewed, and and not assessed in 14 patients (40.0%). Neurofibromatosis pertinent data, including age, sex, tumor location, clinical Type 2 was present in 1 patient (2.9%), absent in 5 patients symptoms, method of treatment, recurrence, duration of (14.3%), and not assessed in 29 patients (82.9%). Eleven follow-up (months), and disease status at final follow-up, cases (30.6%) involved right-sided lesions, and 25 cases were obtained when available. (69.4%) involved left-sided lesions. Gasserian ganglion Exclusion Criteria involvement was reported in 13 cases (36.1%), including those in which the peripheral nerve involvement was not Articles that were not written in English and animal assessed. In total, the distribution of involved trigeminal studies were excluded during the PubMed search. Ar­ nerve branches was not specified in 11 cases (30.6%). Of ticles pertaining to anatomy, anesthesia, biochemistry, the remaining 25 cases for which peripheral branch dis­ natural history, other disease, other tumors, pathology, tributions were specified, 8 (32.0%) had involvement of pharmacology, radiology, and treatments/techniques not
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