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Show J. Clin. Neuro-ophthatmol. 5: 238-243, 1985 © 1985 Raven Press, New York Malignant Optic Nerve Gliomas in Adults A. RUDD, M.R.C.P. J. E. REES, M.D., F.R.C.P. P. KENNEDY, M.D. F.R.C.P. R. O. WELLER, M.D., F.R.C.Path. W. BLACKWOOD, F.R.C.S.(Ed), F.R.C.P.(Ed), F.R.C.Path. Abstract Two patients with malignant optic nerve gliomas are described. The clinical courses and radiographic .ppearances are discussed, with emphasis on the fact that this condition should be considered in the differential diagnosis of rapid visual failure. It can now be strongly suspected preoperatively on the basis of computed tomographic scan appearances. Malignant gliomas of the optic pathways are rare, few having been described since the advent of computerised tomography (CT). The syndrome was first documented in detail by Hoyt et al. l We present two cases that further illustrate the highly malignant nature of the disease and its radiological and pathological appearances. Case 1 A 75-year-old man presented with a 4-day history of progressive loss of vision in the right eye without other symptoms. Visual acuity was hand movement only on the right and 20/30 on the left. There was evidence of an optic nerve lesion with an afferent pupillary defect on the right, but response was normal on the left. Fundal examination was normal. It was impossible to plot central visual fields on the right; the left eye showed a concentrically reduced field to a red target. The erythrocyte sedimentation rate (ESR) was 17 mrnlh. The blood glucose level From the 51. Georges Hospital (A.R.), London, Hurstwood Park Neurological Centre a.E.R. and W.B.), Sussex. Wessex Neurological Centre (P.R.), Southampton, and Southampton General Hospital (R.O. W.), Southampton, England. Write for reprints to: A. Rudd, M.R.C.P., 51. Georges Hospital, Clare House, Blackshaw Rd., London S.W.17, England. 238 and skull x-ray film were normal. A diagnosis of ischaemic papillopathy was made, and the patient was treated with aspirin and vitamin B!2' Within 2 weeks there was no perception of light on the right and vision was 20/60 on the left; over the following week left eye vision deteriorated to 20/200. Pupillary responses indicated optic nerve defects. A repeat ESR was 6 mrnlh. The full blood count, urea and electrolyte tests, blood glucose test, serology for syphilis, cerebrospinal fluid test (CSF), and skull and chest x-ray films were aU normal. Temporal artery biopsy revealed no evidence of inflammation. A CT brain scan (Fig. 1) performed 3 weeks after the initial symptoms showed a possible 1cm contrast-enhancing lesion in the right parasellar area. Tests of visual evoked responses were attempted; there was no discernible response on monocular right or left stimulation to diffuse flash or pattern reversal stimulation. Five weeks after presentation, light could only barely be discerned on the left and a small haemorrhage had become evident in the right fundus close to the disc. Surgical exploration was not considered appropriate in view of the blindness. Despite the lack of evidence supporting a diagnosis of temporal arteritis, high-dose steroids were started., without improvement of symptoms. Over the follOWing 3 weeks, the patient's condition ~ mained stable, but he developed pain in the right eye, suggesting thrombotic glaucoma. The right fundus was studded with haemorrhagel and a few peripheral haemorrhages were visible in the left eye; there was bilateral optic pallor. When the patient was examined 2 weeks later~: frank papilloedema had developed, with a lee sixth nerve paresis, a vertical-gaze palsy (bOtIi up and down), and a right-gaze palsy. ~onvugence was poor. The impairment of conjugate movements was not improved by doll's head manoeuvres, and both plantars were extensor. A repeat CT brain scan was performed 3 Journal of Clinical Neuro-ophthalmoloJn' Figure 1. Cranial cr scan with administration of contrast medium. A possible I-<:m lesion in the right parasellar reo gion is seen. months after the initial symptoms and showed a large, contrast-enhancing mass surrounded by oedema deep in the right cerebral hemisphere extending upward and laterally to the right parasagittal region (Fig. 2). A right temporal burr-hole biopsy revealed astrocytoma, grade m. The patient's condition continued to deteriorate, and he died 4 months after presentation. figure 2. Cranial cr scan with administration of contrast medium showing a large, right·hemisphere mass extending to the parasagittal region. December 1985 Rudd et al. Figure 3. Case 1. Postmortem appearance of brain. g thickened optic nerves and extensive involvement at the right hemisphere. Autopsy revealed a massive, partly necrotic tumour in the right posterior frontal, anterior parietal, lentiform, and thalamic nuclear regions. It extended inferiorly to invade and replace the hypothalamus bilaterally, both cerebral peduncles and the infundibulum. Both optic nerves and the chiasm were involved, and in section they appeared compressed and surrounded by subarachnoid haemorrhagic tissue. Midline structures were displaced to the left (Fig. 3). Neuropathological examination showed a grade III astrocytoma (Fig. 4). Both optic nerves were surrounded by glioma in their subarachnoid spaces (Figs. 5 and 6), were diffusely invaded by glioma, had very congested blood vessels, and were undergoing necrosis. A sleeve of tumour tissue extended along both optic nerves and well into the orbits. Case 2 A 44-year-old woman presented at Southampton General Hospital in August 1979 with a history of deteriorating vision in her right eye over the preceding 6 weeks. There had been slight aching in the eye and she felt generally unwell but was otherwise asymptomatic. On 239 Figure 4. Case 1. Neoplasm in the hypothalamus. Note pleomorphic. hyperchromatic astrocytes with pa1Ii~dingdarou~d the necrosis on the right and capillary glomerular formation on the left. Astrocytoma grade Ill. (Haematoxylin an eosm, x 90). ... - -- - ~ - Flgurt S. Case 1. Portion of the periphery of the right OphC nerve showing dural enveJope on the lower right, ~ vascularized astrocytoma grade III in the middle band. sloping from top right to lower left, and optic nerve largely replaci'4 by neoplasm, parts of which are undergoing necrosis and in which the blood vessels are greatly dilated. (Haematoxylin and eosin, x 90). 240 Journal of Clinical Neuro-ophthalmoleJgY' RuJJ t'l ,11. Figure 6. Case 1. Portion of the periphery of the left optic nerve showing dural envelope on the lower left, astrocytomatous collar in subarachnoid space in the middle band, running from top left to lower right, and optic nerve largely replaced by neoplasm, parts of which are undergoing necrosis and in which the vessels are greatly dilated. (Haemaloxylin and eosin. x 90). presentation there was only light perception in the right eye and a right afferent pupillary defect. The left eye vision was 20/20 and there was a loss of temporal field on the left to a small red target. The right disc was swollen with a few haemorrhages adjacent to it; the left disc appeared normal. A full blood count, urea and electrolyte tests, ESR, blood glucose test, plasma protein levels, autoantibody assays, CSF test, and chest and skull x-ray films and views of the optic canals were all normal. CT brain scan showed diffuse thickening of the right optic nerve throughout its length (Fig. 7). An air encephalogram showed a bean-shaped structure in the suprasellar cistern, and the optiC nerves at the anterior end of the third ven- Figure 7. Case 2. Cranial cr scan with administration of contrast medium shOWing thickened right optic nerve. December 1985 241 Malignant Optic Nerve Gliomas tricle was flattened (Fig. 8). Again, the right optic nerve appeared thickened. Bilateral carotid angiography showed elevation of both anterior cerebral arteries in the frontal view. Visual evoked responses showed prolongation of the latencies in each eye to a flash stimulus, being 170 ms on the left and 200 ms on the right. A clinical diagnosis of adult optic nerve glioma was made and confirmed at operation, when the right optic nerve was found to be replaced by tumour mass extending into the chiasm and left optic nerve (Fig. 9). Much of the tumour showed the features of a well-differentiated astrocytoma, but in some areas, as illustrated in Fig. 9, there was increased cellularity and pleomorphism and increased numbers of mitoses. Although some capillary endothelial proliferation was seen, it was not extensive, and there was no necrosis. A diagnosis of anaplastic astrocytoma was made. The patient died 12 weeks after the onset of the symptoms; there was no autopsy. Discussion Two types of optic nerve glioma are recognised. The commoner form usually presents in childhood, is confined to the optic nerves, and runs a benign course with 85% of patients surviving a mean duration of 17 years. 2 Histology, when obtained, usually shows well-differentiated astrocytoma,3 and there is an association with neurofibromatosis. The more aggressive fo~m, otherwise termed malignant optic nerve glioma, behaves in an invasive fashion, with Figure 8.. Case 2. Air encep~aIogram shOWing bean'shaped st~cture In the suprasellar CIstern and flattening of the right optic nerve. 242 early visual loss and inevitable death. The first recorded case was reported by Collins and Marshall, 4 but Hoyt et al. l were the first to fully define the syndrome of malignant optic nerve glioma of adulthood. They presented five new cases and reviewed 10 previously recorded cases, and suggested that these rare tumours usually occur in middle-aged men, rapidly producing blindness and death within a year. The eight cases reported since by Harper and Stewart-WynneS and Spoor et aJ. 6 combined with our cases suggest that the disease should not be regarded as being confined to middleaged men but should be considered as part of the differential diagnosis of rapid-onset blindness in both sexes at all ages. The two patients described here were blind within 1 month and died at 3 and 4 months after initial symptoms, which emphasises the highly malignant nature of these tumors. Survival of up to 2 years has been reported, but death normally occurs between 6 months and 1 year. No long-term survivors are recorded, and in no case does treat- Figure 9. Case 2. Optic nerve glioma showing an area of Increased cellularity, pleomorphism, and mitotic activity that suggests anaplastic change. (Haematoxylin and eosin, x4(0). Journal of Clinical Neuro-ophthalmology ment seem to have had any significant influence on the disease. Few cases of malignant optic nerve glioma have been reported since the advent of CT brain scanning. In both of our cases, the scan taken in conjunction with the clinical presentation gave a dear indication of the diagnosis and, in case 2, enabled the condition to be diagnosed preoperatively. The CT scan appearances can, however, be misleading. Barbaro et aJ.7 reported a case presenting as a cystic suprasellar lesion, which preoperatively was considered to be a craniopharyngioma or cystic pituitary adenoma. Visual evoked potentials in chiasmal gliomas have been discussed by Kupersmith et al. 8 The cases they discuss are not directly comparable with ours, being apparently less malignant. Nevertheless, their finding of profound changes in visual evoked potentials, particularly to highly textured checker-board pattern stimulus with relatively well-preserved visual acuity, is supported by our findings. The visual evoked potentials in case 2 provided early evidence of severe bilateral optic nerve damage despite 20/ 20 acuity in one eye. The invasive nature of these tumours makes the determination at autopsy of the site of origin difficult, but, taken with the clinical presentation of progressive visual impairment before other neurological deficit, radiological, and electrophysiological findings, there can be little doubt that the tumours arise within the optic nerve and initially spread along the optic pathways. The rapid onset of blindness may result partially from ischaemia of the nerve. This hypothesis is supported by the autopsy findings of congested vessels and necrotic nerve. Prompt diagnosis in cases of rapid visual failure is vital, as many conditions are potentially treatable. Ischaemic papillopathy is frequently the initial diagnosis, as in case 1, but December 1985 Rudd et al. with CT scanning and visual evoked potentials, the diagnosis of malignant optic nerve glioma can be made early in the course of the disease to prevent prolonged invasive investigation in a condition in which the life expectancy is short. Acknowledgment The authors thank Mrs. M. Middleton for typing the manuscript. References 1. Hoyt, W. F., Meshel, L. G., Lessell, S., Schatz, N. J., and Suckling, R. D.: Malignant optic glioma of adulthood. Brain 96: 121-132, 1973. 2. Rush, J. A., Younge, B. R, Campbell, R. J., and MacCartry, C. S.: Optic glioma. Long term follow up of 85 histopathologically verified cases. Am. Acad. Ophthalmol. 89: 1213-1219, 1982. 3. Miller, N. R, Iliff, W. J., and Green, W. R.: Evaluation and management of gliomas of the anterior visual pathways. Brain 97: 743-54, 1974. 4. Collins, E. T., and Marshall, C. D.: Diseases of the optic nerve. Trans. Ophtha/mol. Soc. U.K. 20: 156~ 169, 1900. 5. Harper, C. G., and Stewart-Wynne, E. G.: Malignant optic gliomas i.n adults. Arch. Neurol. 35: 731-735, 1978. 6. Spoor, T. c., Kennerdell, J. 5., Martinez, A. J., and Zorub, D.: Malignant gliomas of the optic pathways. Am. J. Ophtha/mol. 89: 284-292, 1980. 7. Barbaro, N. M., Rosenblum, M. L., Maitland, C. G., Hoyt, W. F., and Davis, R. L.: Malignant optic glioma presenting radiologically as a "cystic" suprasellar mass: case report and review of the literature. Neurosurgery 11: 787-789, 1982. 8. Kupersmith, M. J., Siegel, I. M., Carr, R. E., Ransohoff, J., and Shakin, E.: Visual evoked potentials in chiasmal gliomas in four adults. Arch. Neural. 38: 362, 1981. 243 |
