Cases reported "Optic Nerve Glioma"

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1/33. Malignant optic glioma of adulthood. Case report and review of the literature.

    INTRODUCTION: The malignant optic glioma in adulthood is a rare tumour of middle-age which causes an early loss of vision and always leads to death within a year. CASE REPORT: The authors report a case of this disease in a 68-year-old woman with a history of rapidly deteriorating vision and death 6 months after surgery. CONCLUSION: A review of the previous cases showed the accordance of these with the syndrome defined first by Hoyt et al. in 1973; a statistical analysis reveals that the radiotherapy improves the survival whereas the role of chemotherapy is still not definite. ( info)

2/33. Optic glioma with characteristic bilateral optic atrophy in a 3-year-old girl.

    We report a case of optic glioma with bilateral optic atrophy. A 3-year-old girl presented with vomiting and left hemiparesis. She had hypothalamic dysfunction, right ptosis, right monocular nystagmus, left facial palsy, left hemiparesis, and left pes adductus. neuroimaging studies showed obstructive hydrocephalus with a large suprasellar calcified tumor with a ring-like enhancement mimicking craniopharyngioma. Visual-evoked potentials showed delayed latency of N75 in the right occipital lead. The tumor, a pilocytic astrocytoma in the right optic tract and chiasma, was partially removed via a right frontotemporal craniotomy. The right optic nerve had shrunk to half the normal diameter and became twisted downwardly. intracranial pressure (ICP) increased to 40 cm H2O. The fundus had bilateral optic atrophy without disc swelling. To our knowledge, this is the first report of a lamina/dot sign of the optic disc in a small child with a brain tumor and a normal neuroretinal fiber layer. These ocular findings may result from possible interruption of the axonal flow caused by the tumor and not increased ICP. ( info)

3/33. Outpatient carboplatin desensitization in a pediatric patient with bilateral optic glioma.

    OBJECTIVE: To report a case of successful outpatient carboplatin desensitization in a pediatric patient with bilateral optic glioma. CASE SUMMARY: A 10-year-old white girl with bilateral optic glioma developed a hypersensitivity reaction to carboplatin after nine courses. She had received 18 courses six years earlier without incident. A desensitization regimen was administered, and she has tolerated all subsequent courses of carboplatin therapy. DISCUSSION: carboplatin is an important chemotherapeutic agent in the treatment of a variety of pediatric brain tumors. It is usually given in an outpatient setting. hypersensitivity to this agent appears to develop after frequent exposure such as with the once-weekly regimens often used to treat brain tumors in pediatric patients. CONCLUSIONS: carboplatin desensitization can be successfully performed on an outpatient basis with close observation and cardiorespiratory monitoring. This regimen could prove useful for other patients who develop hypersensitivity reactions to carboplatin and allow therapy to continue. ( info)

4/33. radiation-induced aneurysm and moyamoya vessels presenting with subarachnoid haemorrhage.

    A rare case of a ruptured internal carotid artery (ICA) aneurysm, with ICA stenosis and moyamoya vessels, was diagnosed in a young female patient after radiotherapy for optic glioma. Retrospective analysis revealed that an extraordinarily high dose of radiation (110 Gy) had been delivered to the area around the ICA in two therapeutic sessions. Clipping and wrapping of the aneurysm were performed and the patient made a good recovery. A review of the literature suggested that rupture of radiation-induced aneurysms is a rare event, but may be fatal. Considerable care must therefore be taken when delivering high-dose radiation to cerebral arteries. We suggest that the incidence of radiation-induced moyamoya vessels is higher in younger patients. ( info)

5/33. Spontaneous regression of low-grade astrocytomas in childhood.

    An 8-year-old boy with neurofibromatosis type 1 (NF1) and a biopsy-proven juvenile pilocytic astrocytoma of the hypothalamic/chiasmatic region was followed with serial MRIs over 4 years. Spontaneous tumor regression was followed by progression and biopsy; 6 months later, the tumor regressed again. This bimodal regression is rare, but highlights the variable natural history of low-grade gliomas in children with NF1 and the difficulty in evaluating response of such tumors to therapy. ( info)

6/33. Orbital color Doppler imaging of optic nerve tumors.

    OBJECTIVE: To report changes in retinal arterial and venous blood flow pattern in two patients with tumors involving the entire optic nerve. methods: Retrospective review of one patient with clinical and neuroimaging characteristics typical of bilateral optic nerve gliomas and one patient with a probable meningioma of the left optic nerve sheath. RESULTS: The optic nerve glioma patient had reduced peak systolic velocity of central retinal arteries bilaterally, while the patient with an optic nerve sheath meningioma had relatively low central retinal artery flow velocity and intermittent blood flow in the central retinal vein on the affected side. CONCLUSIONS: Reduced retinal arterial flow velocities in the setting of optic nerve gliomas may correlate with the presence of optic nerve disease. Phasic blood flow in the central retinal vein with optic nerve sheath meningioma may be the reason that some patients with this tumor develop retinal choroidal venous anastomoses. ( info)

7/33. gigantism due to growth hormone excess in a boy with optic glioma.

    True gigantism is rare in early childhood and is usually due to excess GH secretion from a pituitary adenoma. We report a case in which the endocrine abnormality is secondary to an optic glioma. Careful endocrine evaluation has shown that GH peak amplitude was not increased but rather there was failure of GH levels to suppress to baseline and a lack of pulsatility. There is no evidence of a direct secretory role for the tumour and we postulate that the tumour is affecting GH secretion through an effect on somatostatin tone. Specific tumour therapy is not indicated for this patient in the absence of mass effect or visual disturbance. The GH excess is being treated with somatostatin analogue (octreotide) and as he has developed precocious puberty he is also receiving long acting GnRH analogue (Zoladex). This boy appears likely to have neurofibromatosis type 1 (NF1) which raises the question of subtle GH excess in NF1 patients with tall stature. ( info)

8/33. Moyamoya syndrome after radiation therapy for optic pathway glioma: case report.

    We present a 4-year-old girl with neurofibromatosis-1 who developed moyamoya syndrome characterized by bilateral stenosis or occlusion of the distal internal carotid arteries and their branches, leading to the development of an abnormal vascular network. In light of a literature review, the postradiation vasculopathy of the moyamoya type and its relationship with neurofibromatosis-1 are discussed. ( info)

9/33. Neurofibromatosis type 1 and McCune-Albright syndrome occurring in the same patient.

    A patient with both neurofibromatosis type 1 (NF-1) and McCune-Albright syndrome is described. NF-1 and McCune-Albright are separate entities and this is the first report of a patient with clear evidence of both conditions. ( info)

10/33. Extensive gliomas of visual tract in a patient of neurofibromatosis-I.

    Although bilateral optic nerve gliomas are commonly found in patients with neurofibromatosis I, extensive gliomas involving the entire visual tracts, bilaterally are relatively rare. Usually the optic radiations are spared. We report a case of a 2-year-old child with extensive disease of bilateral visual pathways with involvement of the hypothalamus manifesting as obesity. ( info)
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