Cases reported "Optic Nerve Glioma"

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11/33. Spontaneous regression of optic gliomas: thirteen cases documented by serial neuroimaging.

    OBJECTIVE: To demonstrate spontaneous regression of large, clinically symptomatic optic pathway gliomas in patients with and without neurofibromatosis type 1 (NF-1). methods: Patient cases were collected through surveys at 2 consecutive annual meetings of the North American Neuro-ophthalmology Society (NANOS) and through requests on the NANOSNET internet listserv. Serial documentation of tumor signal and size, using magnetic resonance imaging in 11 patients and computed tomography in 2 patients, was used to evaluate clinically symptomatic optic pathway gliomas. All tumors met radiologic criteria for the diagnosis of glioma and 4 patients had biopsy confirmation of their tumors. In 3 patients, some attempt at therapy had been made many years before regression occurred. In one of these, radiation treatment had been given 19 years before tumor regression, while in another, chemotherapy had been administered 5 years before signal changes in the tumor. In the third patient, minimal surgical debulking was performed 1 year before the tumor began to shrink. RESULTS: Spontaneous tumor shrinkage was noted in 12 patients. Eight patients did not have NF-1. In an additional patient without NF-1, a signal change within the tumor without associated shrinkage was detected. Tumor regression was associated with improvement in visual function in 10 of 13 patients, stability of function in 1, and deterioration in 2. CONCLUSIONS: Large, clinically symptomatic optic gliomas may undergo spontaneous regression. Regression was seen in patients with and without NF-1. Regression may manifest either as an overall shrinkage in tumor size, or as a signal change on magnetic resonance imaging. A variable degree of improvement in visual function may accompany regression. The possibility of spontaneous regression of an optic glioma should be considered in the planning of treatment of patients with these tumors. ( info)

12/33. diffusion MRI in neurofibromatosis type 1: ADC evaluations of the optic pathways, and a comparison with normal individuals.

    In a control group of 12 normal children (ages ranging from 9 months to 3 years; mean=1.6 years) the mean apparent diffusion coefficient (ADC) value of the normal white matter, obtained from automatically generated ADC maps, was 0.84 /- 0.14 x 10(-3)mm(2)/s. A patient with neurofibromatosis type 1 with bilateral optic gliomas, and extensive optic pathway involvement was evaluated by diffusion MRI. Multiple measurements of ADC values throughout the involved optic radiations revealed a higher mean value: 1.16 /- 0.06 x 10(-3)mm(2)/s than that of the normal white matter, suggesting relatively high molecular motion in these regions, probably representing myelin vacuolization. ADC evaluation of a thalamic hamartoma revealed a lower value (=1.06 x 10(-3)mm(2)/s) than this. The mean ADC value obtained from multiple measurements of the enlarged optic chiasm, and intraorbital portions of the nerve was similar (=0.81 /- 0.09 x 10(-3)mm(2)/s) to that of the normal cerebral white matter of the control cases. It appears that diffusion imaging can be useful in evaluation of optic pathway involvement in NF1, and might contribute to differentiating optic gliomas from hamartomas, and myelin vacuolization, however, further studies will be required for assessing the role of diffusion imaging in such lesions. ( info)

13/33. neurofibromatosis 1: clinical review and exceptions to the rules.

    neurofibromatosis 1 is an autosomal dominant, multisystem disorder with myriad clinical manifestations. Between 1991 and 1998, 495 adults and children were diagnosed with neurofibromatosis 1 at a specialized neurogenetics clinic in Sydney, australia. This review establishes the prevalence of the clinical manifestations of neurofibromatosis 1 in these patients and provides guidelines for assessment and management. In addition, we review in detail patients who had unusual presentations and who represent important "exceptions to the rules" of clinical practice. ( info)

14/33. A patient with optic pathway glioma, scoliosis, Chiari type I malformation and syringomyelia: is it Neurofibromatosis type 1?

    A 22 years old girl had features of optic pathway glioma, scoliosis, Chiari type 1 malformation and cervical syringomyelia. She had no cutaneous lesions. We considered this combination to be more than coincidental and argue in favour of considering the case as a variant form of Neurofibromatosis type 1. The relevent literature in favour of our contention has been reviewed. ( info)

15/33. Optic pathway gliomas in children with and without neurofibromatosis 1.

    Optic pathway gliomas represent 2 to 5% of brain tumors in children. Frequently asymptomatic, sometimes they demonstrate rapid growth, causing considerable visual dysfunction, neurologic deficits, and endocrine disturbances. Most optic pathway gliomas are diagnosed in patients with neurofibromatosis 1. Little is known about their natural course; therefore, there are no clear and widely accepted guidelines for their treatment. This study compared the clinical manifestations and natural history of sporadic and neurofibromatosis 1-associated optic pathway gliomas with regard to age at diagnosis, gender, and findings on neurologic, ophthalmologic, and neuroradiologic examinations in 83 children with optic pathway gliomas: 51 children with neurofibromatosis 1 and 32 children without any symptoms or signs of neurofibromatosis 1. A prospective study was performed in 21 patients with neurofibromatosis 1. In the rest of the patients with neurofibromatosis 1 and in 32 children with sporadic tumors, the analysis was carried out retrospectively. There was an increased incidence of females in the group of patients with neurofibromatosis 1 with optic pathway gliomas compared with the entire group of patients with neurofibromatosis 1 remaining for follow-up (P = .013). All optic pathway gliomas were found in children below 10 years of age, slightly earlier in the group without neurofibromatosis 1 (median age 4.6 vs 4.8 years). Children with optic pathway gliomas associated with neurofibromatosis 1 had predominantly multifocal lesions (P = .0001), whereas in the group without neurofibromatosis 1, isolated chiasmal involvement was more common (P = .002). Children with sporadic gliomas had significantly more frequently increased intracranial pressure, decreased visual acuity, and abnormalities of fundus of the eye at the time of diagnosis. The radiologic progression, visual deterioration, and endocrinologic complications were documented on follow-up more commonly in children with sporadic tumors. Our findings support the concept that there is an earlier and more severe clinical presentation of optic pathway gliomas in children with sporadic tumors than in those associated with neurofibromatosis 1. ( info)

16/33. optic nerve glioma manifesting as intratumoral hemorrhage in a pregnant woman--case report.

    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. ( info)

17/33. Rapid development of optic glioma in a patient with hybrid phakomatosis: neurofibromatosis type 1 and tuberous sclerosis.

    Increased propensity for tumor formation in neurofibromatosis and tuberous sclerosis exists because of defective tumor-suppressor genes. Although different tumor-suppressor genes may be involved in neurofibromatosis and tuberous sclerosis, at the cellular level these genes share rather common enzymatic pathways. We believe these genetic malfunctions have resulted in a cumulative or additive effect for rapid growth of optic glioma in the following unusual case that has hybrid phakomatosis. ( info)

18/33. Optic radiation involvement in optic pathway gliomas in neurofibromatosis.

    PURPOSE: Optic pathway gliomas (pilocytic astrocytomas) in neurofibromatosis type 1 (NF-1) typically involve some combination of the optic nerves, chiasm, or optic tracts. Involvement of the optic radiations is rare. DESIGN: This paper describes seven patients with NF-1 with gliomas involving the pregeniculate optic pathway in addition to the optic radiations. methods: A retrospective database review was made of all patients with NF-1 and optic pathway gliomas seen by one of the authors (G.T.L.) at the Children's Hospital of philadelphia from July 1993 to October 2001. patients with involvement of pregeniculate optic pathway and the optic radiations were identified. From November 2001 to February 2003, patients were sought prospectively. Cases were also identified from the practice of another author (M.C.B.) at arkansas Children's Hospital. RESULTS: Four patients from Children's Hospital of philadelphia (three of 83 total NF-1/optic pathway gliomas from July 1993 to October 2001 and one prospectively) and three from arkansas Children's Hospital were identified. Two had expanding mass lesions within the white matter of the temporal or parietal lobes, which were histopathologically demonstrated to be pilocytic astrocytomas. The other five had radiographic involvement of the optic radiations but did not undergo biopsy. In three of the cases the vision was 20/200 or worse in each eye. CONCLUSIONS: Optic pathway gliomas in NF-1 may rarely involve the optic radiations. Optic radiation involvement may signal a more aggressive optic pathway glioma in patients with neurofibromatosis-1. ( info)

19/33. Unilateral adult malignant optic nerve glioma.

    INTRODUCTION: adult malignant optic nerve gliomas are rare and rapidly fatal visual pathway tumours. They represent a clinical entity different from the more common childhood benign optic nerve gliomas, which are frequently associated with neurofibromatosis I. CASE REPORT: A 61-year-old woman presented with rapidly progressing right vision loss, lower altitudinal visual field defect and papilloedema. MRI showed intraorbital and intracranial swelling of the right optic nerve. Resection of the intracranial part of the right optic nerve up to the chiasm revealed anaplastic astrocytoma grade III. Within 1 year, the patient died of leptomeningeal metastasis despite radiotherapy. Clinical and MRI evaluation of the left eye and optic nerve were normal at all times. DISCUSSION: Unilateral adult malignant glioma of the optic nerve is exceptional. The final diagnosis was only confirmed by optic nerve biopsy. In the literature, only one patient has been reported with a unilateral tumour manifestation; he was lost to follow-up 3 months later. All other cases were bilateral. To date, 44 case reports of adult malignant optic nerve glioma have been published, either malignant astrocytoma or glioblastoma. These tumours can mimic optic neuritis in their initial presentation. The diagnosis is seldom made before craniotomy. On MRI images, malignant glioma cannot be distinguished from optic nerve enlargement due to other causes. Although radiotherapy appears to prolong life expectancy, all presently available treatment options (radiation, surgery, radio-chemotherapy) are of limited value. Most patients go blind and die within 1 or 2 years. ( info)

20/33. ganglioglioma of the right optic tract: case report and review of the literature.

    We describe a case of ganglioglioma of the right optic tract in a 52-year-old woman. review of the general literature discloses only 11 cases of tumors of the visual pathway that meet the histological criteria for such neoplasm. A detailed radiological description of our case and a review of the literature is provided. ( info)
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