Malignant peripheral nerve sheath tumors of the trigeminal nerve: a systematic review of 36 cases

Abstract

<jats:sec> <jats:title>Object</jats:title> <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></jats:sec> <jats:sec> <jats:title>Methods</jats:title> <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></jats:sec> <jats:sec> <jats:title>Results</jats:title> <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></jats:sec> <jats:sec> <jats:title>Conclusions</jats:title> <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></jats:sec>

Tumores malignos de la vaina nerviosa periférica como origen de dolor orofacial

“…Generalmente no existe predilección por ninguna raza o sexo, pero los TMVNP aparecen comúnmente en pacientes de mayor edad 2 . A pesar de que está ampliamente aceptado que el crecimiento descontrolado de las células de Schwann es la causa de estos tumores, ha sido también planteado que estos pueden aparecer de novo y no surgir a partir de una transformación maligna de un schwannoma benigno [5][6][7][8]10 . La presencia de NF1 aumenta las posibilidades de desarrollar TMVNP; los pacientes con NF1 tienen hasta 4.000 veces más riesgo de desarrollar TMVNP en el transcurso de sus vidas que los individuos que no presentan esta condición.…”

“…A pesar de que la presencia de NF1, en general, aumenta las posibilidades de aparición de TMVNP, es interesante hacer notar que esta asociación no ha demostrado tener un rol significante en los TMVNP de los nervios craneales 5,6 . Los TMVNP constituyen del 2 al 6% de los sarcomas de cabeza y cuello 10 . Se le ha atribuido al género un pequeño rol en el desarrollo de TMVNP intracraneales: a diferencia del resto de TMVNP, tienen una alta incidencia reportada en hombres.…”

“…Se le ha atribuido al género un pequeño rol en el desarrollo de TMVNP intracraneales: a diferencia del resto de TMVNP, tienen una alta incidencia reportada en hombres. Una revisión sistemá-tica reciente de TMVNP trigeminales corrobora esta idea, ya que los autores encontraron un 77,1% de pacientes hombres en su análisis, con una edad promedio de 44,6 años y un rango de 8 a 71 años 10 .…”

“…Se ha planteado que la afectación de más de una rama es característica de los TMVNP que se originan en el ganglio de Gasser, permitiéndoles su expansión a las múltiples divisiones del nervio 10 . Sin embargo, no todos los TMVNP trigeminales se originan necesariamente en el ganglio de Gasser; también se ha evidenciado que los TMVNP trigeminales se extienden de divisiones posganglionares mas allá del ganglio de Gasser.…”

“…Considerando los signos clínicos de los TMVNP trigeminales, estos pueden ser difíciles de diagnosticar con base en las manifestaciones clínicas, ya que pudieran asemejarse a las de un schwannoma benigno del nervio trigémino [6][7][8][9][10] . La revisión de la literatura en inglés muestra una gran variedad de síntomas reportados.…”