Intracranial optic nerve angioblastoma.

Stefani, F. H.; Rothemund, Elisabeth

doi:10.1136/bjo.58.9.823

Cited by 23 publications

(12 citation statements)

References 1 publication

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“…Despite the frequent association of CNS hemangioblastomas with VHL disease, optic nerve hemangioblastomas have been reported in only 9 cases of VHL disease (mean age at discovery 33.4 ± 15.0 years) in the English literature, including the present case 4–7,14,17–19. Although 1 optic nerve hemangioblastoma was discovered at autopsy, 9 tumors had been resected from 8 patients (1 patient with bilateral tumors; Table 1).…”

Section: Discussionmentioning

confidence: 76%

Progressive peritumoral edema defining the optic fibers and resulting in reversible visual loss

Baggenstos

Chew

Butman

et al. 2008

JNS

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Hemangioblastomas are frequently associated with peritumoral edema caused by extravasation of plasma ultra-filtrate through permeable neoplastic vessels. The authors report the clinical and imaging findings in a 62-year-old man with von Hippel–Lindau disease who presented with rapid (within 24 hours) loss of color vision and near-complete loss of left eye vision (acuity too poor to test). Serial MR imaging demonstrated a stable vascular tumor in the medioinferior aspect of the left optic nerve, associated with progressive edema extending from the nerve through to the bilateral optic radiations. Complete resection of the lesion was performed via an extended transsphenoidal approach, and histological examination confirmed the lesion was a hemangioblastoma. Postoperatively, the patient recovered color vision and had improvement in visual acuity (20/320). Serial imaging in this unique case captured the progressive extravasation of peritumoral edema that tracked and defined the parallel white matter tracts of first- and second-order neurons of the optic system, causing vision loss. Tumor resection led to resolution of the edema and improvement in visual function.

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Section: Discussionmentioning

confidence: 76%

Progressive peritumoral edema defining the optic fibers and resulting in reversible visual loss

Baggenstos

Chew

Butman

et al. 2008

JNS

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“…Surgery was performed in all cases except one. 16) The visual function was preserved after surgery in several patients. 3,8,13) All patients with optic nerve hemangioblastoma suffer progressive visual disturbance leading to blindness, so total removal in the early stage is recommended.…”

Section: Discussionmentioning

confidence: 91%

“…2,4,7,9,11,13,17) These findings suggest that hemangioblastoma arises from the optic nerve tissue rather than the optic nerve sheath, 3,5,12) and may be useful to differentiate hemangioblastoma from meningioma and schwannoma. Histological examination shows that optic nerve hemangioblastoma usually does not have a capsule, 9,16,17) in contrast to meningioma. Immunohistochemical analysis reveals negative immunoreactivity of stromal cells to EMA, also different from meningioma.…”

Section: Discussionmentioning

confidence: 99%

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Hemangioblastoma of the Optic Nerve-Case Report-

Higashida

Sakata

Kanno

et al. 2007

Neurol. Med. Chir.(Tokyo)

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A 64-year-old man presented with a rare sporadic hemangioblastoma arising in the left optic nerve manifesting as left visual disturbance gradually progressive over 5 years. Magnetic resonance imaging revealed a well-enhanced mass in the left optic nerve. Partial resection of the tumor was performed via the frontoorbital approach. The histological diagnosis was optic nerve hemangioblastoma. Hemangioblastoma must be considered in the differential diagnosis of optic nerve tumors even in the absence of other lesions associated with von Hippel-Lindau disease.

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“…Darr et al (1966) give references to other cases in the literature. Although optic nerve hemangiomas have a predilection for the optic disc, they may also occur within the orbital or intracranial optic nerve (Stefani & Rothemund, 1974).…”

Section: Angiomatous Tumors and Malformationsmentioning

confidence: 99%

Tumors of the optic nerve

1976

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This is a review of primary and secondary tumors of the optic nerve. The emphasis is an optic nerve gliomas and meningiomas. Optic nerve gliomas are slowly growing astrocytic neoplasms of the anterior visual pathways, the majority of which occur within the first two decades of life, with equal sex incidence in about 1 of 200,000 patients presenting with eye complaints. The incidence is greater in neurofibromatosis. The typical presentation is visual impairment in a verbal pre-school child with optic canal enlargement and optic atrophy. An intraorbital location of the tumor leads to axial, irreducible, non-pulsatile proptosis. An intracranial location may disturb hypothalamic and pituitary function and produce hydrocephalus. Ocular findings may also include limited motility on a mechanical-restrictive basis, a pupillary relative afferent defect, nystagmus, and variable, non-specific visual field defects. Roentgenographic studies may show concentric unilateral enlargement of the optic canal with preservation of a well corticated margin, a fossa under the anterior clinoid process in continuity with the optic canal ('J'-shaped sella), and findings of increased intracranial pressure. On pathologic examination the tumor is a smooth, fusiform, intradural enlargement of the optic nerve. Histologically there is proliferation of elongated (pilocytic) astrocytes in reticulated patterns with intervening microcystic spaces containing mucosubstance and surrounding reactive hyperplasia of the arachnoid. Mitoses are not found. The diagnosis is clinical X-ray studies and brain scan should be performed. The differential diagnosis is that of unilateral proptosis in a child and includes acute ethmoiditis, hyperthyrobidism, craniostenosis, other neoplasms, Hand-Schuller-Christian disease, and orbital hemorrhage due to trauma. Surgical resection is performed in cases with unilateral optic nerve involvement, the surgical approach being determined by tumor location. Bilateral or chiasmal cases are treated with radiotherapy when progression occurs. Malignant optic nerve gliomas and optic nerve hyperplasia are also discussed. Optic nerve meningiomas arise from the nerve sheath and are to be distinguished from orbital meningiomas arising from ectopic arachnoidal cells or those secondarily involving the orbit by extension from adjacent sites. Up to 80% of orbital meningiomas occur in females, in two age peaks, 25% in the first decade, and the rest in the 5th decade. Meningiomas present with visual loss and may produce proptosis, papilledema and/or optic atrophy, retinal striae, opticociliary shunts, limitation of extra-ocular movements, and lid edema, Signs of von Recklinghausen's disease should be sought. X-rays are the mainstay of diagnosis. Orbital meningiomas are composed of cells in sheets or in whorls with some spindle shaped cells. Calcifications are typical. Usually the dura is penetrated and the orbit invaded. Primary orbital meningiomas are locally infiltrating but do not metastasize. Complete local excision en bloc is recommended...

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Intracranial optic nerve angioblastoma.

Cited by 23 publications

References 1 publication

Progressive peritumoral edema defining the optic fibers and resulting in reversible visual loss

Progressive peritumoral edema defining the optic fibers and resulting in reversible visual loss

Hemangioblastoma of the Optic Nerve-Case Report-

Tumors of the optic nerve

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