Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0017638 (glioma)
 30,880 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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.
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PMID:Recurrent Optic Nerve Pilocytic Astrocytoma: A Rare Case. 2865 22

Neurofibromatosis type 2 (NF2) is a rare genetic disorder, affecting the central nervous system and leading to various degrees of disability. Its hallmark is bilateral vestibular schwannomas that invariably lead to progressive hearing loss. Specific ophthalmic abnormalities in patients with NF2 may help to establish an early diagnosis. These include juvenile cataract, epiretinal membrane, combined hamartoma of the retina and the retinal pigment epithelium, optic disc glioma, and optic nerve sheath meningioma. In addition, intracranial tumors may produce a variety of neuro-ophthalmic abnormalities that have the potential to impair visual function, such as postpapilledema optic atrophy, compression of the visual pathways, keratopathy, ocular motor cranial nerve palsies, and amblyopia. Care of NF2 patients is best provided by interdisciplinary medical teams including a neuro-ophthalmologist.
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PMID:The Role of Neuro-Ophthalmologists in the Care of Patients With Neurofibromatosis Type 2. 3279 46