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Query: UMLS:C0017638 (glioma)
 30,880 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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

An anaplastic glioma of the optic nerve, involving the globe, optic chiasma and brain in a 3 1/2-year-old Labrador Retriever is described. The tumour consisted of lobules of small, dark cells intersected by a delicate fibrovascular stroma. There was a high degree of anaplasia and an average of 19 mitoses per x 200 field. The clinical signs were exophthalmos and mydriasis, followed several months later by blindness and, only terminally, by signs of brain involvement.
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PMID:Glioma of the optic nerve with intraocular and intracranial involvement in a dog. 355 6

The clinical information on 22 patients with orbital optic nerve gliomas and 47 patients with meningiomas was correlated with computed tomographic findings obtained in both axial and coronal studies. Most of the gliomas occurred in children, although 7 patients presented after 20 years of age. Among the patients with meningiomas, the majority were women in early middle age, although two tumors occurred in children less than 20 years of age. Low grades of proptosis (median, 2 mm for both tumors), frequent significant visual field obscurations with eye movements, and opto-ciliary shunt vessels pointed toward the diagnosis of an optic nerve tumor. Patients with gliomas generally manifested massively swollen fusiform optic nerves with clear-cut margins due to circumscription by an intact dura. Kinks and bucklings of the optic nerve as well as infarctive cysts distinguished the glioma CT-scan patterns from the meningiomas. Distinctive axial CT-scan features of the meningiomas not shared by the gliomas were narrowly and diffusely enlarged nerves with polar expansions either at the orbital apex or immediately behind the globe; calcification; irregular excrescent margins signifying extradural invasion into the orbital soft tissues; a negative optic nerve shadow running down the center of the lesion; and bone erosion near the orbital apex. Coronal studies often revealed irregular margins signifying transgression of the dura. A diffusely and narrowly enlarged optic nerve shadow with regular margins (intrasheath lesions) was the one morphologically overlapping pattern displayed by 11 meningiomas and three gliomas. In these cases there tended to be more profound visual loss in the gliomas compared with the meningiomas, as well as the more frequent presence of opto-ciliary vessels in the meningiomas. Arteriography may be helpful in this particular category by demonstrating a tumor blush for the meningiomas, whereas this finding is typically absent with optic nerve gliomas. Meningiomas may be very closely simulated by dural or intraneural inflammations.
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PMID:Combined clinical and computed tomographic diagnosis of orbital glioma and meningioma. 670 28

Report on an 18-year-old man with a histologically confirmed intraorbital meningioma (transitional type) of the right optic nerve. The symptoms were slowly progressive loss of vision with central scotoma and pallor of the disk, without proptosis or disturbance of eye motility. Computer tomography performed preoperatively did not permit the differential diagnosis of glioma.
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PMID:[Symptomatology of a primary intraorbital meningioma of the optic nerve]. 713 92

Juvenile pilocytic astrocytoma of the optic nerve (glioma of childhood) usually grows slowly. Rapidly evolving proptosis is rare and reflects the accumulation of mucinous or hemorrhagic material within the tumor. Rapid proptosis is more common in those patients beyond the first two decades of life who have long-standing tumors. We studied a 26-year-old woman with rapid proptosis caused by a large blood-filled cyst in a pilocytic astrocytoma of the optic nerve. The cyst was surgically removed along with the intraorbital portion of the optic nerve.
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PMID:Pilocytic astrocytoma of the optic nerve with hemorrhage and extreme cystic degeneration. 730 96

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

Gliomas of the anterior visual pathway are rare orbital lesions accounting for 65% of all intrinsic optic nerve tumors. These lesions occur principally among children in the first decade of life. They appear to be true neoplasms that characteristically show early growth, followed by stability in many patients. Visual prognosis is fair, and the outlook for life depends upon tumor location. When initially confined to the optic nerve alone, overall mortality is about 5%. Once the hypothalamus is involved, mortality rises sharply to over 50%. With involvement of the chiasm or hypothalamus, no form of therapy significantly alters the final outcome. Because of their indolent course, gliomas may be followed conservatively when confined to the optic nerve. In these cases, surgery is indicated only when blindness and pain or severe proptosis intervene. However, all such patients should be followed radiologically for evidence of posterior extension. When the chiasm is threatened, surgical excision via a craniotomy approach is warranted to prevent subsequent hypothalamic or third ventricle involvement. Malignant optic glioma is a distinct disease primarily affecting middle-aged adults. The chiasm is always involved, and although one eye may be spared initially, rapid progression to bilateral blindness is usual. The disease is uniformly fatal.
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PMID:Gliomas of the anterior visual pathway. 800 27

A 4-month-old infant came to the department of Ophthalmology, King Chulalongkorn Memorial Hospital with right eye proptosis, strabismus, and no vision. She was diagnosed as optic nerve and chiasm glioma. The subtotal removal of the tumor was performed and followed by chemotherapy, with a satisfactory result. Because of the large size of the tumor and the presenting symptoms and signs since the patient was only 2 month old, we believe that this tumor originated in the intrauterine period. To our knowledge, this reported case is the youngest patient with optic nerve and chiasm astrocytoma in Thailand.
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PMID:Optic nerve glioma in infancy: a case report of the youngest patient in Thailand. 1152 26

A 51-year-old male presented with a 5-year history of progressive right exophthalmos and visual loss. Magnetic resonance imaging showed a right intraorbital lesion. [(18)F]fluorodeoxyglucose positron emission tomography ([(18)F]FDG-PET) revealed high uptake in the tumor. No FDG was accumulated in the remaining optic tract. He underwent removal of the tumor. The histological diagnosis was optic glioma. Six months after the operation, the tumor recurred, and a second operation was performed to spare the visual acuity of the other eye, which remained stable for 1.5 years after the first operation. However, the patient died 2 years after the first operation of liver dysfunction. Adult optic glioma tends to rapidly extend into the surrounding tissue and to affect the contralateral visual pathway. Early diagnosis and surgical intervention are imperative. In the present case, [(18)F]FDG-PET was valuable for evaluating malignancy to determine whether radical surgery was indicated to preserve the remaining visual acuity.
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PMID:Surgical decision for adult optic glioma based on [18F]fluorodeoxyglucose positron emission tomography study. 1706 90

Optic pathway glioma (OPG), seen in 15% to 20% of individuals with neurofibromatosis type 1 (NF1), account for significant morbidity in young children with NF1. Overwhelmingly a tumor of children younger than 7 years, OPG may present in individuals with NF1 at any age. Although many OPG may remain indolent and never cause signs or symptoms, others lead to vision loss, proptosis, or precocious puberty. Because the natural history and treatment of NF1-associated OPG is different from that of sporadic OPG in individuals without NF1, a task force composed of basic scientists and clinical researchers was assembled in 1997 to propose a set of guidelines for the diagnosis and management of NF1-associated OPG. This new review highlights advances in our understanding of the pathophysiology and clinical behavior of these tumors made over the last 10 years. Controversies in both the diagnosis and management of these tumors are examined. Finally, specific evidence-based recommendations are proposed for clinicians caring for children with NF1.
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PMID:Optic pathway gliomas in neurofibromatosis-1: controversies and recommendations. 1738 25


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