RESUMEN
Schwannomas are the fourth most common primary neoplasms affecting the brain and cranial nerves. Central lesions commonly arise from sensory nerve roots, and a common intracranial site is the vestibular branch of the 8th nerve (>85%). We present the case report of a patient who has a schwannoma extending from the pterygopalatine fossa to the orbit, complaining about facial pain in the trajectory of the trigeminal ophthalmic branch. Schwannomas represent 1 to 2% of all neoplasms of the orbit, and trigeminal schwannomas are extremely rare, accounting for less than 0.5% of all intracranial tumors.
Asunto(s)
Humanos , Femenino , Persona de Mediana Edad , Neoplasias de los Nervios Craneales/cirugía , Enfermedades del Nervio Trigémino/cirugía , Neurilemoma/cirugía , Órbita , Neoplasias de los Nervios Craneales/radioterapia , Neoplasias de los Nervios Craneales/diagnóstico por imagen , Neurilemoma/patología , Neurilemoma/diagnóstico por imagenAsunto(s)
Adenocarcinoma/secundario , Neoplasias de los Nervios Craneales/secundario , Enfermedades del Nervio Oculomotor/etiología , Nervio Oculomotor , Neoplasias de la Próstata/diagnóstico , Adenocarcinoma/diagnóstico , Adenocarcinoma/radioterapia , Adenocarcinoma/cirugía , Anciano , Neoplasias de los Nervios Craneales/diagnóstico , Neoplasias de los Nervios Craneales/radioterapia , Neoplasias de los Nervios Craneales/cirugía , Humanos , Hipofisectomía , Imagen por Resonancia Magnética , Masculino , Orquiectomía , Neoplasias de la Próstata/radioterapia , Neoplasias de la Próstata/cirugía , Agudeza Visual , Campos VisualesRESUMEN
The association between neurofibromatosis and visual pathway gliomas is well documented. The introduction of computed tomography and magnetic resonance imaging has heralded a new era in the understanding of visual pathway gliomas. Both of these noninvasive neuroinvestigative techniques have demonstrated extensive abnormalities throughout the visual pathway in children with visual pathway gliomas, especially in those with neurofibromatosis. The clinical significance of these abnormal areas of brain, especially in asymptomatic patients, is unknown. In an attempt to clarify the incidence, natural history, and clinical course of patients with neurofibromatosis and visual pathway lesions, we reviewed our experience with 24 patients managed consecutively at Children's Hospital of Philadelphia over the past 12 years. The patients in this series were compared to 29 children with visual pathway gliomas without neurofibromatosis who were evaluated at our institution over the same period of time. Visual pathway gliomas in children with neurofibromatosis differ from those in children without neurofibromatosis. In general, lesions tended to be more extensive in patients with neurofibromatosis and the clinical course of these patients is more variable. Twelve of the 24 patients with neurofibromatosis in our series had symptoms of progressive disease at the time of diagnosis and underwent treatment with variable results. Twelve children with neurofibromatosis and visual pathway lesions had static lesions at the time of diagnosis and, to date, 3 have developed progressive disease. From our review we can make some recommendations concerning the management of children with neurofibromatosis and visual pathway gliomas, but many questions remain unanswered. Sequential follow-up of a large cohort of both asymptomatic and symptomatic children with neurofibromatosis and visual pathway lesions is needed to more definitively outline the best management approach for these patients.