Cases reported "Optic Nerve Neoplasms"

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1/111. color Doppler image of central retinal artery of eyes with an intraconal mass.

    PURPOSE: Retinal ischemia secondary to hypoperfusion of the central retinal artery is recognized as one factor that may contribute to the development of loss of vision in eyes with intraorbital tumors. We study intraorbital tumors which produce motility disturbances and visual problems by color Doppler imaging to evaluate this factor. methods: We examined the central retinal artery velocities of 3 patients with disc edema caused by intraconal masses (2 cavernous hemangiomas and 1 presumed optic nerve glioma) via color Doppler imaging. RESULTS: The time-velocity waveform demonstrated abnormally high vascular resistance in the central retinal artery of all affected eyes in the primary position compared with the normal waveform seen in the other eyes. We compared the pulsatility index of eyes with an intraconal mass and contralateral, control eyes using Student's t test for paired samples and significant differences were noted between both groups (p< 0.01). CONCLUSIONS: Intraconal tumors could produce increased pressure in the optic nerve sheath and the optic nerve tissue which could be associated with impaired retinal and optic nerve blood flow and the subsequent amaurosis encountered with intraorbital tumors. ( info)

2/111. indocyanine green angiography of optic nerve head melanocytoma.

    PURPOSE: To determine the features of fluorescein and indocyanine green angiography of melanocytoma. methods: Fluorescein and indocyanine green angiography is used to assess an optic nerve head melanocytoma in a 45-year-old female. RESULTS: fluorescein angiography revealed increased vascularity on the surface, with staining around the lesion in the late stages. The lesion was hypofluorescent in all stages of indocyanine green angiography. CONCLUSIONS: indocyanine green angiography is helpful in identifying the benign nature of the lesion by showing hypofluorescence, indicating lack of vascularity in the tumor. ( info)

3/111. Cavernous hemangioma of the intracranial optic pathways: CT and MRI.

    PURPOSE: The purpose of this work was to describe the CT and MR findings in three patients with cavernous hemangioma (CH) of the intracranial optic pathways. METHOD: CT and MR studies of three patients with CH of the optic chiasm were reviewed. All patients underwent MRI of the chiasmal area, with coronal T2- and T1-weighted studies as well as gadolinium-enhanced coronal and sagittal T1-weighted studies. RESULTS: The patients (mean age, 40 years) presented with chiasmal apoplexy (two cases) and progressive decrease of visual acuity (one case). In all cases, MRI showed regular enlargement of the optic chiasm, with extension to the optic nerve in one case and to the left optic tract in one case. The chiasmatic dimension was 2.5-3 cm in two cases and 1-1.5 cm in the other case. In all cases, MRI revealed an acute (isointense signal on T1-weighted and hypointense signal on T2-weighted sequences) or subacute (hyperintense signal on T1 - and T2-weighted sequences) hemorrhage with, adjacent to it, an area with signals of blood of different ages, highly suggestive of CH. CT showed, in chiasmatic CHs, a suprasellar mass spontaneously denser than adjacent brain parenchyma. In two cases, microcalcifications were associated. In two cases, CT and MRI revealed slight heterogeneous enhancement after contrast agent administration. In one case, no enhancement was observed. Two patients underwent surgery by frontopterional craniotomy. The optic chiasms were swollen with an intrinsic bluish mass. The cerebrospinal fluid was not xanthochromic. Microscope examination confirmed the diagnosis of CH. After 12 months, the operated patients had improved visual acuity and visual field but did not completely recover. The nonoperated patient (because of spontaneous rapid recovery of visual acuity) was followed clinically and on MRI over 18 months. CONCLUSION: CH in the optic chiasm must be suspected in the presence of an acute chiasmatic syndrome. MRI is the best imaging modality, showing either an acute or a subacute chiasmatic hemorrhage or the typical pattern of CH with heterogeneous alternation of foci of blood of different ages, with a central focus of methemoglobin, a peripheral rim of hemosiderin, adjacent foci of acute or subacute hemorrhage, and slight or no enhancement after gadolinium administration. ( info)

4/111. Malignant transformation of an optic disk melanocytoma.

    PURPOSE: To report a case of malignant transformation of an optic disk melanocytoma with a second melanocytoma in the ciliary body. methods: Clinical data including visual acuity, visual fields, color fundus photographs, fluorescein angiogram, and ultrasonogram and histopathologic studies of this case were reviewed. RESULTS: The right eye of a 65-year-old white woman was diagnosed with melanocytoma of the optic nerve. Four years later, the tumor became significantly larger. The best-corrected visual acuity declined from 20/40 to counting fingers and the size of the tumor increased fourfold in 2 years. The right globe was enucleated. Histopathologic studies demonstrated moderately pigmented spindle-B malignant melanoma cells adjacent to and within a population of large, polyhedral, heavily pigmented melanocytoma cells that extended to the lamina cribrosa and optic nerve. There was also a deeply pigmented melanocytoma in the ciliary body. CONCLUSION: This is a rare case of malignant melanoma transformed from an optic disk melanocytoma. Periodic follow-up of the patient with optic disk melanocytoma is necessary. ( info)

5/111. Spontaneous partial regression of low-grade glioma in children with neurofibromatosis-1: a real possibility.

    At the age of 41 and 31 months, respectively, a boy and a girl affected by neurofibromatosis-1 were diagnosed with a visual pathway glioma during surveillance contrast-enhanced head magnetic resonance imaging (MRI). In the first child, the initial MRI showed that the entire optic chiasm, the intracranial tract of the left optic nerve, and hypothalamus were grossly enlarged and enhanced in the post-gadolinium T1-weighted images. Ten months later, the hypothalamic component of the lesion had regressed markedly and there were no more areas of contrast enhancement. In the second child, the initial MRI showed that the optic chiasm, the right optic tract, and geniculate body were enlarged and enhanced after gadolinium injection. At 6-month follow-up, the MRI showed that the right optic tract and the anterior aspect of the optic chiasm decreased in size and the contrast enhancement of the entire lesion was reduced dramatically. These findings, as indicated by other similar reports, confirm that spontaneous regression of visual pathway glioma is a rare but real possibility in children with neurofibromatosis-1. Therefore, clinicians need to be aware of visual pathway glioma's erratic behavior in children with neurofibromatosis-1 with special attention given to the importance of a very conservative attitude toward any type of treatment for such patients. ( info)

6/111. Extraocular extension of unrecognized choroidal melanoma simulating a primary optic nerve tumor: report of two cases.

    BACKGROUND: Orbital extraocular extension of choroidal melanoma is well known and is usually detected in eyes with medium and large tumors, but it is very rare with small melanomas. It is particularly unusual for choroidal melanomas of any size to invade the optic nerve or its meninges. DESIGN: Two case reports. PARTICIPANTS: Two patients with small, relatively inconspicuous juxtapapillary pigmented choroidal lesions were referred with the diagnosis of primary optic nerve tumor. Both demonstrated a large nodular tumor in the meninges of the optic nerve, immediately posterior to the globe. methods: Retrospective review of clinical records and histopathology. RESULTS: In both cases, orbital magnetic resonance imaging confirmed the presence of a hyperintense enhancing nodular mass near the anterior portion of the optic nerve, prompting optic nerve biopsy in one case. Subsequent fundus examination disclosed a small juxtapapillary pigmented choroidal lesion measuring 1.0 mm or less in thickness. These observations suggested that the optic nerve tumor might be nodular extraocular extension of a small choroidal melanoma. Modified enucleation was performed in both cases, and histopathologic examination revealed a nodule of malignant melanoma within the meninges that compressed the optic nerve and extended extraocularly from a small, relatively inapparent juxtapapillary choroidal melanoma. In both cases, the extraocular component was large and symptomatic, whereas the intraocular component was inconspicuous. CONCLUSIONS: Small juxtapapillary choroidal melanomas can exhibit prominent extension into the orbit. All patients with orbital tumors should have careful ophthalmoscopy. ( info)

7/111. A new approach to an old problem.

    A patient with progressive visual loss was found to have an optic nerve sheath meningioma. The patient was treated with stereotactic radiotherapy, a computer-guided stereotactic technique that minimizes the risk of radiation-induced optic neuropathy. Six months after treatment, the patient was doing well and showed no signs of radiation-induced optic neuropathy. ( info)

8/111. Recurrent NF1 gene mutation in a patient with oligosymptomatic neurofibromatosis type 1 (NF1).

    We report a 21-year-old male with symptomatic optic glioma who does not fulfill the diagnosis of neurofibromatosis 1 (NF1) according to standard NIH criteria. Analysis of the NF1 gene revealed a recurrent mutation in exon 37 (C6792A or Y2264X). This nonsense mutation causes skipping of exon 37 during the splicing process and is predicted to result in a protein shortened by 34 amino acid residues. The mutation was detected in all tissues examined (blood lymphocytes, oral mucosa, and dermal fibroblasts). The same mutation was previously found in 3 patients with clinically confirmed NF1. To our knowledge, this is the first report of an adult patient carrying a putative (non-mosaic) NF1 gene mutation in multiple tissues but not fulfilling the NIH criteria for the clinical diagnosis of NF1. ( info)

9/111. Solitary metastasis of breast carcinoma in the optic chiasm.

    Optic chiasmatic compression from suprasellar metastatic lesions is well known, but metastasis intrinsic to the optic chiasm has not been reported. A 45-year-old woman, with treated breast carcinoma, presented with headache and chiasmatic syndrome from a large suprasellar tumour. At surgery, an exophytic chiasmatic tumour was encountered, with an appearance similar to a glioma. The pathological appearance was consistent with the primary neoplasm in the breast. ( info)

10/111. Spontaneous improvement of optic pathway lesions in children with neurofibromatosis type 1.

    Two cases of spontaneous improvement of optic pathway lesions in neurofibromatosis type 1 are reported. At time of diagnosis the children were aged 21 and 32 months respectively; they have been followed by both MRI and clinical evaluation for 5 and 4 years. MRI findings of the first 19 months of follow-up for Case 1 have been described by us before. On MRI serial evaluation, Case 1 showed an almost complete normalization of the size of lesions and a resolution of enhancement, whereas Case 2 showed a slight decrease in the size of lesions and a resolution of enhancement. From the clinical point of view Case 1 showed a normalization of his clinical signs, whereas in Case 2, a visual improvement was only slight, if at all present. In Case 1, the clinical improvement seemed to follow the spontaneous regression of the lesions detected by MRI. ( info)
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