RESUMEN
Retinal hemangioblastomas are one of the most common and early manifestations of Von Hippel-Lindau disease. Early detection is the key in their management. When left untreated, these benign neoplasms may continue to grow and result in scleral infiltration and extraocular extension warranting enucleation of the globe.
Asunto(s)
Neoplasias Cerebelosas/complicaciones , Hongos/fisiología , Hemangioblastoma/complicaciones , Neoplasias de la Retina/complicaciones , Enfermedades de la Esclerótica/patología , Adulto , Neoplasias Cerebelosas/patología , Neoplasias Cerebelosas/terapia , Terapia Combinada , Femenino , Hemangioblastoma/patología , Hemangioblastoma/terapia , Humanos , Pronóstico , Neoplasias de la Retina/patología , Neoplasias de la Retina/terapia , Enfermedades de la Esclerótica/etiologíaRESUMEN
Pilocytic astrocytoma is a low grade glioma that affects mostly children and young adults and can occur anywhere in the central nervous system. Pilocytic astrocytoma of the optic nerve is an equally indolent subtype that is often associated with Neurofibromatosis Type I (NFI). A 40-year-old male presented with left sided axial proptosis and exposure keratopathy. MRI revealed a mass in left proximal orbit, extending posteriorly abutting the chiasma and the right optic nerve on MRI. Enucleation of the left eye along with near total excision of intracranial part of the mass was performed. Histopathology report was suggestive of pilocytic astrocytoma (WHO Grade I). Interestingly, his records showed evidence of surgery for removal of the optic nerve pilocytic astrocytoma twice (27 years and six years ago). We hereby, present an unusual case of recurrent pilocytic astrocytoma of the optic nerve in absence of NFI.
