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Richard F. Schmidt, Frederick Yick, Zain Boghani, Jean Anderson Eloy and James K. Liu

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.

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Richard F. Schmidt, Zain Boghani, Osamah J. Choudhry, Jean Anderson Eloy, Robert W. Jyung and James K. Liu

With the relatively recent increase in the use of MRI techniques, there has been a concurrent rise in the number of vestibular schwannomas (VSs) detected as incidental findings. These incidental VSs may be prevalent in up to 0.02%–0.07% of individuals undergoing MRI and represent a significant portion of all diagnosed VSs. The management of these lesions poses a significant challenge for practitioners. Most incidental VSs tend to be small and associated with minimal symptoms, permitting them to be managed conservatively at the time of diagnosis. However, relatively few indicators consistently predict tumor growth and patient outcomes. Furthermore, growth rates have been shown to vary significantly over time with a large variety of long-term growth patterns. Thus, early MRI screening for continued tumor growth followed by repeated MRI studies and clinical assessments throughout the patient's life is an essential component in a conservative management strategy. Note that tumor growth is typically associated with a worsening of symptoms in patients who undergo conservative management, and many of these symptoms have been shown to significantly impact the patient's quality of life. Specific indications for the termination of conservative management vary across studies, but secondary intervention has been shown to be a relatively safe option in most patients with progressive disease. Patients with incidental VSs will probably qualify for a course of conservative management at diagnosis, and regular imaging combined with the expectation that the tumor and symptoms may change at any interval is crucial to ensuring positive long-term outcomes in these patients. In this report, the authors discuss the current literature pertaining to the prevalence of incidental VSs and various considerations in the management of these lesions. It is hoped that by incorporating an understanding of tumor growth, patient outcomes, and management strategies, practitioners will be able to effectively address this challenging disease entity.