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)

Optic nerve gliomas were diagnosed with computed tomography (CT) in 10 children. CT: (A) provides the best radiographic means of demonstrating the orbital portion of the optic nerves; (b) can evaluate the clinically unsuspected optic nerve for bilateral gliomas; (c) demonstrates gross intracranial involvement; and (d) can be used to follow the course of a glioma. Gliomas can present on CT as (a) a uniform thickening of the entire nerve; (b) a solitary fusiform enlargement of the nerve; or (c) an irregular solid thickening along the nerve. The authors routinely use axial CT of optic nerves as the primary diagnostic scan.
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PMID:Computed tomography of intraorbital optic nerve gliomas in children. 69 2

The authors present a case of an optic nerve hemangioblastoma in a young woman with von Hippel-Lindau disease. The initial diagnosis was made by incisional biopsy. Tumor growth led to progressive proptosis and loss of light perception. Excision was carried out by lateral orbitotomy. Clinically and radiographically, the tumor resembled an optic nerve meningioma or glioma. Review of the other known cases offers no information as to the potential spread of this benign tumor from the intraorbital optic nerve to the optic canal. Optic nerve hemangioblastoma must be considered in the differential diagnosis of optic nerve tumors in patients with or without von Hippel-Lindau disease.
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PMID:Hemangioblastoma of the optic nerve. Report of a case and review of literature. 305 Jun 87

Fifty-three patients with neurogenic orbital tumors were diagnosed and treated at our university hospital during the past three decades. There were 16 patients with juvenile pilocytic astrocytomas, 21 patients with meningiomas and 16 with peripheral nerve tumors. Of the 16 peripheral nerve tumor patients, 8 had schwannoma, 4 had neurofibroma, 2 had malignant schwannoma, one had paraganglioma and one had amputation neuroma. Optic nerve tumors were treated via a transfrontal craniotomy if there was no chiasmal involvement. Cases with chiasmal involvement, incomplete resections and recurrences were treated with radiotherapy (5000 cGy). Far advanced cases with progressive proptosis and ocular damage underwent exenteration. Peripheral nerve tumors were treated by local resection. Exenteration was applied when there was an advanced or malignant lesion. Four of the 16 glioma patients and 5 of the 21 meningioma patients died during follow-up. The prognosis for peripheral nerve tumors was generally good, except for malignant schwannomas. Two patients with this malignant tumor died within 2 years after surgery.
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PMID:Neurogenic tumors of the orbit. 796 11

Optic nerve gliomas may occur alone or as components of neurofibromatosis. They are rare tumors accounting for 2 to 5% of all intracranial tumors and 6% of the intra-orbitary neoplasms. The authors present 11 cases of optic nerve-glioma diagnosed in Curitiba in the last 25 years. Out of these 11 patients there were 10 women and only 1 man. The ages ranged from 3 to 25 years old, and 6 patients were under 15 years. In this series, 27.3% (n = 3) of the cases were associated with neurofibromatosis. Five patients had their tumors restricted to the optic nerve while the others had either extension to the optic chiasm, supra-selar region, frontal or temporal lobe. All the patients had pilocytic astrocytomas.
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PMID:[Optic nerve gliomas: a study of 11 cases]. 898 86

Optic nerve gliomas and meningiomas, which remain puzzling to clinicians, are reviewed with special attention to natural history, magnetic resonance findings, and appropriate management. New histopathological insights established the true neoplastic nature of optic nerve gliomas considered by most clinicians to be benign hamartomas. Unusual presenting manifestations and therapeutic alternatives of optic glioma have been also considered. Since the association of optic nerve gliomas and meningiomas with neurofibromatosis has long been recognized, special attention has been drawn to clinical, genetic, and ophthalmologic aspects of neurofibromatosis. New and unusual presenting manifestations, diagnostic and treatment opportunities concerning optic nerve involvement in metastatic disease, and lymphomatous proliferation are also described.
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PMID:Tumors of the optic nerve and chiasm. 1015 Aug 23

A 33-year-old pregnant woman presented with intratumoral hemorrhage in an astrocytoma of the left optic nerve and chiasm manifesting as poor vision in her left eye. Visual examination found no light perception in the left eye and deteriorated acuity with temporal hemianopsia in the right eye. Computed tomography demonstrated a round high-density mass in the suprasellar region. T2-weighted magnetic resonance (MR) imaging showed the center of the mass as mildly hypointense and T2-weighted MR imaging as hypointense. Both T1- and T2-weighted MR imaging showed the rim of the mass as hyperintense. She delivered a healthy baby by cesarean section. A left frontotemporal craniotomy was then performed. Incision of the lateral surface of the left optic nerve revealed clotted blood from the left optic nerve and the left side of the chiasm. No vascular malformation was noted in the hematoma cavity. Histological examination revealed a neoplasm composed of well-differentiated astrocytic cells. No Rosenthal fibers were identified. The patient made an uneventful postoperative recovery. Her right visual acuity returned to normal, but the temporal hemianopsia in the right eye and the blindness in the left eye persisted. Gliomas of the anterior visual pathways are rare, especially in adults. The hemorrhage may have been caused by the hypercoagulable state associated with pregnancy. Optic nerve astrocytic glioma with intratumoral hemorrhage should be considered in the differential diagnosis of suprasellar hematoma.
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PMID:Optic nerve glioma manifesting as intratumoral hemorrhage in a pregnant woman--case report. 1470 24

Optic pathway glioma associated with neurofibromatosis 1 has a classically indolent course. However, involvement of the optic radiations is relatively rare and is associated with a more aggressive course. A three-year-old girl presented with strabismus and loss of vision in the left eye with relative afferent pupillary defect and optic disc pallor. She had multiple cafiota au lait spots. Visually evoked potential was suggestive of an optic nerve conduction defect and magnetic resonance imaging of the brain was suggestive of an optic pathway glioma involving the optic nerves, the optic chiasma and the optic tracts. The optic radiations and the dendate nuclei had hamartomas. Optic nerve biopsy confirmed pilocytic astrocytoma. Radical radiotherapy under general anesthesia was subsequently given. This case report aims to highlight the involvement of the optic radiations and the unusually aggressive clinical course in this case.
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PMID:A case of neurofibromatosis 1 presenting with optic pathway glioma with an early onset and an aggressive course. 1829 34

Primary optic nerve gliomas are most commonly benign pilocytic astrocytomas (World Health Organization [WHO] Grade I) occurring in childhood and following an indolent course. Malignant optic gliomas occur in adulthood and follow an extremely aggressive course, with rapid infiltration of the chiasm, blindness, and death typically within months. A third category of optic glioma, occurring in adulthood, histopathologically benign (WHO Grade I-II) but following an aggressive course, has been rarely reported. The authors describe clinical and histopathologic features of clinically aggressive but histopathologically benign optic nerve gliomas of adulthood. Retrospective review of cases of biopsy-proven optic nerve glioma in the neuro-ophthalmology division of the Jules Stein Eye Institute from 1990 to 2011 was carried out. Cases following an aggressive course were selected for review of clinical, neuroradiologic, and histopathologic features. Three cases were selected for detailed study. Ages ranged from 31 to 45 years. All were initially diagnosed with optic nerve inflammation or benign neoplasm based on clinical and neuroradiologic features, but all suffered neuroradiologic extension and rapid deterioration of vision in the affected eye to no light perception over 3-8 weeks. Optic nerve biopsies were undertaken for the suspicion of malignancy. Features ranged from WHO Grade I (pilocytic astrocytoma, ganglioglioma) in two cases, to WHO Grade II in one case (diffuse astrocytoma, histopathologically benign, but associated with aggressive features such as high p53 [13-21%] and Ki-67 [40%]). The diffuse astrocytoma case subsequently developed extensive intracranial extension suspicious for malignant transformation. These findings indicate that benign optic nerve glioma in adults may be initially misdiagnosed as inflammation, be clinically aggressive, and require excision to prevent further intracranial involvement.
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PMID:Aggressive Low-Grade Optic Nerve Glioma in Adults. 2792 17

Optic nerve glioblastoma is a rare entity that usually presents with rapidly progressive vision loss, which eventually results in blindness and, ultimately, death. As with malignant gliomas in other anatomical locations, local recurrence is common. Isolated rapid changes in vision, atypical neuroimaging findings, and the rarity of optic nerve glioblastoma may render diagnosis challenging and, thus, delay treatment. The authors present a case of optic nerve glioblastoma that was treated with subtotal resection followed by adjuvant radiation therapy and temozolomide. One year following the initial diagnosis, the patient developed a right cerebellar lesion, which was histopathologically consistent with glioblastoma. This case represents the first report of transtentorial dissemination of an optic nerve glioblastoma. In addition, the authors reviewed the literature regarding optic nerve glioblastomas. Of the 73 previously reported cases of malignant optic nerve gliomas, 32 were histologically confirmed glioblastomas. The mean age at diagnosis was 62 years, and 56% were male; the median survival was 7 months. A malignant glioma of the optic nerve should be considered in the differential diagnosis of a patient with rapidly progressive visual loss. However, the incidence of optic nerve glioblastoma is exceedingly low.
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PMID:Transtentorial dissemination of optic nerve glioblastoma: case report. 2829 17

Optic nerve pilocytic astrocytoma is an uncommon but well-known entity; however, intraorbital ancient pilocytic astrocytoma of the optic nerve is extremely rarely reported. To our knowledge, this is the first detailed description regarding the intraorbital ancient pilocytic astrocytoma, reported in available English literature, to date. We presented an extremely unusual neurofibromatosis type 1 case of a 17-year-old male's sudden ocular pain secondary to intraorbital pilocytic astrocytoma of the optic nerve with markedly cystic degeneration, fluid production, and hemorrhage, due to ancient and possibly ruptured glioma. Future prospective studies are required to validate the significance of intraorbital ancient pilocytic astrocytoma arising from the optic nerve and the close correlation with ruptured cystic degeneration and ocular pain, after collecting and investigating a larger number of its cases examined.
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PMID:Intraorbital ancient pilocytic astrocytoma of the optic nerve in neurofibromatosis type 1 patient presenting with sudden ocular pain. 2951 46


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