Journal of Neuro-Ophthalmology, March 1981, Volume 1, Issue 1

Chiasmal optic glioma after radiation therapy. Neuro-ophthalmologic/pathologic correlation.

A 16-year-old white girl with neurofibromatosis was documented as having progressive visual loss in both eyes over 3 years before diagnosis of a chiasmal glioma. She was then treated with supervoltage irradiation to the sella and parasellar area. Bitemporal fields measuring 5 x 5 cm each were initially used, and source skin distance of 80 cm with coplanar opposing technique was used whereby each field was treated daily to a midplane dose of 186 rads. The patient received a cumulative tumor dose over the 5-week course of 4680 rads. She tolerated this well, and her visual function was stabilized thereafter for over a year. Two years following therapy, she expired suddenly and unexpectedly at home. At autopsy, there was no radionecrosis in the brain or optic nerves, but minimal radiation changes were seen in the tumor. Extensive local invasion was seen in the entire chiasm, adjacent optic tracts and hypothalamus. In addition, other disparate small fibrillary astrocytic gliomas were found in the optic radiations, midbrain, and left anterior midfrontal lobe. This patient therefore documents clinically progressive visual deterioration before irradiation therapy, and palliative visual function stability for well over a year after irradiation therapy. The patient also demonstrates the difficulty in treating a locally aggressive optic chiasmal glioma, its infiltrative nature, and the multifocality of other unsuspected neural tumors in neurofibromatosis.

]. Oin. N.·uro-ophthalmol. 1: 31-43, 1981. Chiasmal Optic Glioma after Radiation Therapy Neuro-ophthalrnologic/Pathologic Correlation JOSEPH C. PARKER, JR., M.D. J. LAWTON SMITH, M.D. PATRICIO REYES, M.D. MARIO M. VUKSANOVIC, M.D. Abstract A l6-yeu-old white girl with neurofibromatosis was documented .IS having progressive visual loss in both eyes over 3 years before diagnosis of a chiasmal gUom... She was then tre..ted with supervoltage irra­diation to the sella imd parasellar area. Bitemporal fields measuring 5 x 5 cm each were initially used, and source skin distance of 80 em with coplanar op­posing technique was used whereby each field was treated daily to a midplane dose of 186 rads. The p.ltient received a cumul..tive tumor dose over the 5­week course of 4680 rads. She tolerated this well, and her visuill function was stabilized thereafter for over a yeu. Two years following therapy, she expired sud­denly ilnd unexpectedly at home. At autopsy, there was no r..dionecrosis in the br..in or optic nerves, but minimal radiation changes were seen in the tumor. Extensive local inv..sion was seen in the entire chiasm, adj..cent optic tracts and hypo­th< l1amus. In addition, other disp..rate small fibrillary astrocytic gliomas were found in the optic radiations, midbrain, ilnd left ..nterior midfrontal lobe. This pa­tient therefore documents diniully progressive visual deterioration before irr..diation therapy, and palliative visual function stability for well over .. year ..Eter irra­di.. tion therapy. The patient ..Iso demonstrates the dif­ficulty in tre..ting a loc..lly aggressive optic chiasma! g1iom.., its infiltr..tive nature, and the multifoulity of other unsuspected neural tumors in neurofibromato­sis. AJthough gliomas of the anterior visual pathway have been reported as "hamartomatous" lesions with insignificant aggressive behavior and meta- From the Departments of Pathology and Ophthalmology, Uni­versity of Miami School of Medicine, and the Department of Therapeutic Radiology, Cedars of Lebanon Hospital. Miami, Florida. March 1981 static potentiaL I prolonged follow-up of these usu­ally young individuals has at times demonstrated their locally invasive nature at autopsy.2 Because of the differences in these reports, clinical contro­versy has existed in recent years among advocates of observation, radiation therapy, and surgery as the best management for optic gHomas. A partic­ularly instructive case is therefore presented of a 16-year-old girl with neurofibromatosis treated with radiation therapy fOI a chiasmal glioma in whom detailed historical, neuro-ophthalmologic, radiologic, and pathologic documentation is avail­able. Case Report A 13-year-old, right-handed, white girl was first seen at the Bascom Palmer Eye Institute on Decem­ber 16, 1975, through the courtesy of Dr. Richard Brown. The chief complaint was poor vision in the right eye. She was the product of a normal preg­nancy, but was delivered by cesarean section after a 6-hour labor trial. At birth a notched right upper lid anomaly was noted. This was felt to be an insignificant cosmetic defect, and an ophthalmo­logic plastic consultant saw her at age 4 and advised against surgical repair. Growth and development were normal, and annual screening vision tests in school were said to be normal. At 10 years of age, a pediatrician noted a vertical strabismus in her right eye. An ophthalmologist then saw the patient, and recorded her visual acuity in February 1972 as 20/50-2 in the right eye and 20/25+2 in the left eye. The left eye was patched without success for a short time, and indeed not only did the right eye vision not respond, but over the next 2 years the vision in her right eye dropped further. There were no headaches or other neurologic complaints. Be­cause of the steady painless visual deterioration, she was referred for neuro-ophthalmologic evalu­ation. 31 Chiasm,ll Optic GliomJ ,litpr RJdiation Thprapy External E amindtion EXJmination in December 1975 revealed a cor· rected Clcuity of 20/400 and 7.0 M in the right eye, dnd 20/50-2 Jnd )-1 in the left eye. External ex­.1minJtion reveJled J notched right upper lid (Fi~. I) ,Jnd subtle pigmented lesions on her neck (Fig. 2). The Idlter were associJted with subcutaneous nodules which were eJsily pJlpable on her neck, left deltoid dreJ Jnd left lower rib cage. A few smClII pigment spots were also seen on her back. A right hypertropia due to a left Brown's superior oblique tendon sheath syndrome was present. The pupils were normal. A dense bitemporal hemiano­pia was evident (Fig. 3). The optic discs were pale in both eyes, more so on the left. Jndirect ophthal­moscopy revealed a peripheral subretinal neurofi­broma in the right eye. One look at the patient's father (Fig. 4) disclosed multiple IClrge cutaneous fClcial lesions typical of 32 Figure 1. Cl'n~c.·nll,,)J .lnvnlJlv \.If rlKnt upper lid .\.\.'tC" lhE\' n~t-u Mvpl'nr\1r,..) J ..~,I(IJI d \\ Ith Brl1\Vn'.. "upCrll1r ",~IH.fU(' tend,,'n she.uh ~vndr(\m(' In thl$ pJtl~nt figure 2. !'>ubllp p",nlPnlJry eh.nVA tn 'f n " ,," un p npe~" I 'S fldhPnl. Subcutdneous nodules were PJ"Iy pJlpJl>I,· on Ihi' JIPd. Journal of Clinical Neuro-ophthalmology opr.'-f_J_U _ 01" "_'_'"_'_/'_' _ 1".... ••~••h )/]:)0 vtJlu o mE ile Mi." VltaJ F'ln..D, I).. tl~" ..i'.~ "'••• .a.'1 I~. 10/1'0 1i 1~-'"' Int lit AI.-tll: \\ 10/400 0 I') "1 yllL-.. _ Figu,~ J. Visual fi..ld ....min.tion prior to irr.di.tion tht>rapy. Figur~ 4, Th.. I.th .. r·s I.ciall... tu,... typic. I of n..urofibromatosis. von Recklinghausen's neurofibromatosis and im­mediately established the diagnosis, Furthermore, he reported that his mother also had had the identical appearance! March 1981 Further communication with previous examiners was then made and documented the visual acuities in the patient as seen in Table 1. The clinical diagnoses were: multiple neurofibromatosis with 33 ChiJsmal Optic Glioma Jfter Radiation Therapy chiasmal glioma; right congenital upper lid colo­boma; subretinal neurofibroma, right eye; and Brown's tendon sheath syndrome, left. Routine skull films were obtained and were negative but for d congf'nital Harlequin appearance of thf' orbits. Optic foramen vif'WS were symmet­ricdl Jnd revf'aled 4 X 5 mm canals in both eyes on the Rhese projections. An EMI scan and a repeat delta scan with enhancemf'nt were both reported as normal. Pneumoencephalography was then per­formed by Dr. Yates in January 1976 and rf'vealed an enlarged and thickened optic chiasm. The pa­tif'nt W,lS treated by irradiation therapy to the chiasm by Dr. Vuksanovic, and received a cumu­lative tumor dose of 4680 rads given to the selJa and parasellar region over the 5-week interval of February 11 through March 17, 1976. Subsequent visual acuities are seen in Table 2. If one compares both the distance and near acuities, it will be recalled that the patient had 20/ 400 and 7.0 M in the right eye and 20/50-2 and J-TABLE 2. Visual Acuities After Irradialion Therapy" 2·11-76 through 3-17-76 20/400 20/60+1 14/200 20/70 15/200 20/50 14/200 20/80 14/200 20/80+2 TABLE I. Previous Visual Acuities Before Irradiation Therapy n.ne RL~nl Ey(" VISIon left Lye VI~lon Feb.IQn 20/50·2 20/25+2 lune 1972 20/70 20/25 July 1972 20/50 20/25-3 Sept. 1972 20/50 20/25 Dec. 1975 20/400 20/50+1 12-16-75 Radiotherapy 2-24-76 3-16-76 6-15-76 8-26·76 1-4-77 • Recorded by )LS. Righi E),t" Vision 20/400 Leh Eye Vision 20/SO-1 'tHO .,.,.,It.e T40',L ~1Io1.~h ""_1l VlSU.AiL. FIELD. O"nl.i.'.~ Du.__-=-!-~-7;.;6 _ Tn..., r...:1.+ 2. ~ OS 't' U'Ut: 34 ("mhlC.T11 UIlAStt C:LIOMA IN NtUf!OFtfUlOP1ATnSIS .- ~D 10th RADIOnU:.IlAPV U.£ATtENT TODAY I'i\) Cll!WCE. IN VISLOrt QM n£LOS TO nils POINT Figure s. VisUJI fi~ld eXJminati"n during irradiation Iherapy. Journal of Clinical Neuro-ophthalmology rJrlwr, SmIth. «.,yt·s. Vuks~novi a••,.,.-, _J.111~''--- _ 1.,.. 1·\ 1200 iI:.t.l~rd~~ lJIf!:tA.Il914 ntBlAfj IhCl'I 2'.U.J~ thl"'Ji ] ... 17.16 to M'll. iliil\d Pllt .....llu· .n.... C::HI.uM Gl.l.OJ1IIl VI$UAL P'1u.,ce t.... ,loilit .J1/:1].Ou...r&Ilil!: HI 1nt:] ,",...,.•••• ,.10 p_r4o!:IiM:r.! iPU'l,...ra.! h ••d' U Mil. dlc-" ta JI )IJ III ~. 1M1)" IO/JJC) I 4<'1'1* T ... " •• J••I~ lJ));t MN..'" (01 .c: ilta.rk 1i)J );~ r-d. (C) _1IIIi ,.~ flgu .... 6. V'''WlI fidd ~umln.h()11 .l ."d of "r.di~lIon therap 0,..,.,... _....IIL;I=.. _ Dliitl _.;.1_.':..:'_"':..:1 _ Cot 5'J2Ob 'h1•• fLo Nt If)30 ~th 10) - l r U<l,U:f :lIC AJiiII.rl:. ,..rl_l.r ,.....~..~ .. -+ )/])0 YnLt.. (Uo) J!JJO ~lu (0) • \Jut ..! Lu~",t:... VI15UAL fl'IELD-S T.u ."J..b'--;.r;;~ilt'l.'.n. [i..2....(..,j.i ...- 1I1la (nJ- ~"nn (...,I,......J,l ho:ptu LZ AI. [ "hlotl .nd r l.ld..a ullib 1e dlJrffli Hrw.t ' ....T .r'hr 1f*;JI(lC h il!T*W 1 in the left eye on December 16, 1975-before irradiation therapy-and was found to have 14/ 200 and 3.0 M in the right eye and 20/80+2 and J­1 in the left eye on January 4, 1977-9 months after irradiation therapy. Visual field examinations during, at the end of, and 10 months after radio-therapy are seen in Figures 5-7. The patient dem­onstrated improvement in Amsler grid pattem in the left eye following radiotherapy, and subjec­tively reported better vision. It was considered after reviewing distance and near acuities, Amsler grid patterns, and serial visual field examinations that March 1981 35 Chiasmal Optic Glioma after Radiation Therapy Postmortem examination A complete autopsy examination was performed by Dr. Walker B. Sorrell, who kindly provided all of the tissues reviewed for this report. The general autopsy examination revealed moderate pulmo- TABLE 3. Visual Acuities After Irradiation Therapy· although the patient's visual function had been decreasing during the 3-year interval prior to irra­diation therapy, that her visual function had sta­bilized for over a year following therapy. She was then followed by her ophthalmologist, Dr. Richard Brown, ilt home who kindly provided the acuities presented in Table 3. It should be noted that all of the acuities recorded in this report are the best corrected acuities. The patient did well in general after radiotherapy, and resumed her school and other activities. Two and a half years after the diilgno is of chiasm.:!1 glioma, on April 3, 1978, the patient died suddenly and unexpectedly at home. • Kindly prOVided by Dr. R. E. Brown. nary edema and biventricular cardiac dilatation. Scattered over the body were small cafe-au-Jait spots, especially evident on the neck and chest. Enlarged, ropy, white-tan, myxoid plexiform pe­ripheral nerves were scattered through the body, particularly in the neck. Multiple sections of these enlarged abnormal nerves demonstrated typical pleXiform neurofibromata diagnostic of von Reck­Iinghausen's neurofibromatosis (Figs. 8a and 8b). No other significant abnormalities were recognized in the thoracic or abdominal viscera. The central nervous system included a well-fixed brain that weighed 1190 g_ Both eyes and optic nerves were included in toto with the brain (Fig. 9). The most striking extemal abnormality was noted in the optic chiasm which was markedly distorted by an enlarged, white, firm mass measur­ing 2 X I X 1 em (Figs. 10 and 11). Adjacent proximal segments of the right and left optic nerves were atrophic and measured 0.3 em in diameter (Fig. 12). The optic tracts were involved adjacent to the chiasm (Fig. 13) but appeared pale white and shrunken a short distance distally, and measured 0.4 em in diameter. All cerebral vessels were intact and grossly normal. The cerebral hemispheres were cut coronally at I-em intervals. A coronal cut is seen in Figure 14 (left side) through the level of the anterior chiasm. In Figure 15 (upper right side) a coronal cut is seen through the posterior chiasma! level. Note that the entire left basal ganglion region is enlarged and 20/70 20/60 20/60 20/60 20/60 20/70 Lt-(e Eye VISion 20/400 20/400 .F. .F. .F. CF. oa2.3-7tJ 11.20 -70 J·I-77 0-19-77 1-11-78 3-21-78 D.He rol' Ilrmll'1J11I1I11fI~jurJ .~ ~-'-?'~...... (./o~/" h(;un· ... U" II> .dl ,,<J '•• Ir n ~'~rsc <;('(l1un d~m, mtrat<'S lh~ It.1 ht~ wilh .II t·1n'nt ffi)lxord df'N1.I1("rd.. w... (' I' rm n('Uf fJbrQrndta dl~flU'),ht uf VUI1 ,...- II~h.u,,·n· n('url'flbtl'l'ndtD!>L. ~ dif(u.~ rny~oid pte 'form neuro(j. brom.l (flro"t .id,·J " dis" t"u~ d. J6 Joumal of Clinical Neuro-ophthalmo!ogy Figur~ 9. External view of brain with enlarged and distorted optic chiasm with attached atrophic proximal optic nerves. March 1981 distorted by an infiltrative astrocytic neoplasm in direct continuity with the chiasm. Notice the com­pressed left lateral and third ventricle and the adjacent swollen centrum semiovale. A more rostral coronal section revealed another discrete lesion in this case. This was a 3 X 3 X 0.5 em, discrete, tan-brown mass involving leptomen­inges, cortex and falx cerebri, around the left me­dial inferior and anterior frontal lobe, and extended to the genu of the corpus callosum (Fig. 16). A small, tan, 0.5-cm lesion was also seen in the left anterior internal capsule. The optic radiations were grossly normal. The brainstem and cerebellum were intact. The spinal cord was not available. Multiple sections through the brain and brain­stem were obtained for light microscopy. The neo­plasm in the optic chiasm (Fig. 17) and inferior hypothalamus was characterized by multiple, atyp­ical, fibrillary astrocytes with scattered microcystic changes, irregular nucleomegaly, focal nucleoli, and scattered Rosenthal fibers with focal hemosid­erin and malignant gemistocytes. The proximal segments of both optic nerves in their intracranial portions demonstrated diffuse demyelination, scat­tered calcospherites, and focal irregular nucleo­megaly consistent with polypoid nuclei of chrcmic radiation injury (Figs. 18 and 19). Within the in­ferior mid-frontal region, atypical fibrillary astro­cytes were associated with scattered hemosiderin, malignant gemistocytes, and Rosenthal fibers that involved the leptomeninges and adjacent cortex. 37 ChiasmaI Optic Glioma after Radidtion Therapy Figure 10. The ,'pti chiJ<m in the coronal brain.section (left side, is severt'ly expanded by an infiltrating fibrillary astrocytoma. An .Id,."ent n<'rmal uptic ch'Jsm (rollht s.de) IS provIded for comparison. Figure 11. The optic chiasm is demyelinated and expanded by a dIffusely infiltrative fibrillary astrocytoma. The reduced cellularity of the glioma in some areas is compativle with previoos radiation effect. (H&tE/LFB. X4.) This lesion was distinct and separate from the optic chiasmal glioma and failed to reveal any radiation changes. Small foci of atypical fibrillary astrocytes were seen as discrete microaggregates in the left and right optic radiations, subpial area of the cere­bral peduncle, right hypothalamus, and left mid­inferior frontal lobe. "Hamartomatous" multinu­cleated astrocytes were seen in some tumor foci. The medulla, pituitary, and pons were unremark­able. Focal Purkinje cell loss with reduced myeli­nation was evident. Within the left anterior hip­pocampal region, there were telangiectatic vessels 38 with gliosis, focal lymphoid cells and hemosid­erosis consistent with chronic radiation changes. Ectopic glial tissue with neurons was seen in the left anterior internal capsule consistent with a ham­artoma- normal mature tissue that is abnormally arranged, but in a normal location. In summary, this 16-year-old girl had docu­mented von Recklinghausen's neurofibromatosis with multiple cervical plexiform neurofibromas, cdfe-du-ldit spots, and an extensive well-differen­tiated fibrillary astrocytoma (not a hamartoma) involving both proximal optic nerves, the optic Journal of Clinical Neuro-ophthalmology March 1981 Figure 12. M~lInified IIr,,", e~tern.1 photograph showing intracr.nidl optic nerves ~b,we. with ~trophir s"gm"nts, dnd enlarged and thick"ned optir chiasm below. Figur. 13. The thickening e~tends into optic trarts as seen in this figur". ;1" ~ 1'"I"~"~ , Figur. 14. The left sid" shows a coronal cut of this pati"nt's brain at an anlerior chiasmalleveL A normal brain is on the right side for comparison, 39 Chiasmal Optic GIi(lm" after Radiation Therapy Figure 15. Coronal ections of this p.tient's brain. The upper right picture is • posterior chidsm level. Note that the enlire left basdl ganglion regIon IS enlarged dnd dIStorted by tumor in direct continuIty wilh the chidsm. Also nole thdt left Idler.1 dnd third ventricle are compressed .nd note the swollen white matter in the ddjacent centrum semiovdle. Figure 16. Leptomeningeal dstracytic infiltrate anses from the cortex in tht." inrt'riur dnterior medidl frontdl .Ired, 40 chiasm, both optic tracts, and the adjacent hypo­thalamus. The fact that the intracranial segments of both optic nerves were quite atrophic within about 5-7 mm from the chiasm showed that this was a true chiasmal glioma, and did not arise in the optic nerve and secondarily extend into the chiasm. In addition, and as expected in a patient with the central variety of heurofibromatosis, mul­tiple, small fibrillary astroeytic gliomas with focal hamartomatous, cytomegalic features involved the optic radiations, midbrain, and left anterior mid­frontal lobe. Although isolated suggestive chronic radiation changes were seen in the proximal optic nerves, there was no evidence of radionecrosis in the brain or visual pathways. Despite the fact that the patient's visual deteriordtion had stabilized for over a year after radiation therapy, at autopsy the patient had persistent invasive astrocytoma in the hypothalamus and disparate unsuspected astrocy­toma in other areas of the I.entral nervous system. Although the immediate cause of death was not apparent morphologically, the biventricular cardiac dilatation with pulmonary edema in an individual dying unexpectedly implies cardiac arrthymias. In this young girl with neurofibromatosis and dem­onstrated peripheral nerv" lesions, her cardiac death seemed neurally mediated. Journal of Clinical Neuro-ophthalmology March 1981 Puker, Smith. Reyes, Vuksanovic Figu.... 17. Th oplic chi••m .nd .dl.cenl hypothAlAmus dre infiltrAled by Awell·differentiAted fibrill.ry Astrocytom. wilh scoHered RosenthAl fibers (Ihe dArk round mAsses). (H&E/lfB. xI50.} .. figure 1&. Crv» ,.,hon vI onl .., ..no.1 portoon Ollhos optIC nerve reve.ls reve.ls diffuse cenlr.1 demyelln.tion And gliosis. No tumor is seen. (H&E/lfB, X4.) 41 Chi.lsmal Optic Gliom.l Jfter RJdi.llion Therapy ~ Figure 19. longitudinal sections of the posterior optIC nerve segments adjdcent to the chid~m possess focdl calcifications (left lower dred). sCdttered ghosis. dnd demyehnahon conSIstent with chronic radldtion changes. No tumor is seen. (H&E/LFB. X50.' Comment The distinction between the slow-growing neo­plasm that has local infiltrative properties and may even degenerate to a frankly anaplastic malig­nancy- from a benign, well-encapsulated neo­plasm with no malignant potential-is important clinically and pathologically. Kernohan and Sayre3 classified gliomas with the well-differentiated (grade I) astrocytic tumors as part of a spectrum of malignant gliomas, varying from the least aggres­sive grade I glioma to the most aggressive grade IV glioblastoma multiforme. At autopsy, a well-dif­ferentiated brainstem glioma frequently possesses atypical fibrillary astrocytes similar to those seen in the optic chiasmal glioma; however, the brain­stem glioma at autopsy contains foci of glioblas­toma multiforme. This phenomenon rarely, if ever, occurs in the anterior visudl pathway. I. •. 10 Never­theless, well-differentiated fibrillary astrocytomas anywhere in the central nervous system have a relentlessly locally aggressive behavior, which may be associated in some regions of the brain, brain­stem, and spinal cord with more malignant features developing histomorphologic patterns of poorly differentiated gliomas like the glioblastoma multi­forme, the primitive neuroectodermal tumor, or both, some weeks or months later. Well-differen­tiated fibrillary astrocytomas throughout the cen­tral nervous system are compatible with prolonged survival measured in years, unlike the poorly dif- 42 ferentiated glioblastoma multiforme with survival measured in weeks to months. A hamartoma represents a cellular mass of ap­parent congenital rest origin composed of normal mature tissue that is abnormally arranged but in a location in which the tissues are expected.~ Well­differentiated fibrillary astrocytomas do not con­tain normal tissue, for these astrocytic elements appear morphologically and biologically to behave as abnormal cells. In addition, the associated mi­crocystic changes and scattered Rosenthal fibers so commonly seen in gliomas of the anterior visual pathways are abnormal morphologic features that are not compatible with the hamartomatous nature thdt some have claimed for these tumors. I. J The term "hamartoma'" is therefore inappropriate for an optic chiasmal glioma, which should be consid­ered a neoplastic glial lesion with the potential to expand and infiltrate adjacent tissues over months to years. The well-recognized infiltrative properties characteristic of well-differentiated malignant gliomas in the central nervous system can separate yet maintain the integrity of neuritic tracts, pro­ducing relatively little neurological deficits with their large tumors. Finally, it is clinically important to differentiate between gliomas arising within the optic nerves, and gliomas arising within the chiasm. The case here reported was a pure chiasmal glioma in that andtomic confirmation of focal atrophy of the in­tracranial portions of both optic nerves was evident Journal of Clinical Neuro-ophthdlmology (Fig. 12). In our opinion, optic gliomas should be treated as gliomas. This statement would apply to both optic nerve glion.as and optic chiasm gliomds. We do not address ourselves in this pdper to the proper clinical management of gliomas of the optic nerves, and feel that those cases must be carefully evaluated on an individual basis. However, with regard to gliomas of the optic chiasm, we do not feel that chiasmdl gliomas are SUrgiCdl candidates, but we agree with Taveras and Mount6 and Miller et al." that they may be palliated after rddiotherdpy. References 1. Hoyt, W.F., and Baghdassarian, S.A.: Optic glioma of childhood. Natural history and rationale for con­servative management. Br. I. Ophthd/mo/. 53: 793­798, 1969. 2. Aarabi, B., long. D.M., and Miller, N.R.: Enlarging optic chiasmal glioma with stable visual acuity. Surg. Neuro/. 10: 175-177, 1978. 3. Kernohan, J.W., and Sayre, G.P.: Tumors of the central nervous system. In At/ds of Tumor Pathol­ogy, fase. 35. Armed Forces Institute of Pathology, Washington, D.C., 1952. 4. Glaser, J.5., Hoyt, W.F., and Corbett, 1.: Visual mor­bidity with chiasmaI glioma. Arch. Ophtha/mol. 85: 3-12,19n. 5. Robbins, S.l., Angell, M.: Basic Pdthology (2nd ed.). W.B. Saunders, Philadelphia, 1976. 6. Taveras, I.M., and Mount, l.A.: The value of radia­tion therapy in the management of glioma in optic nerves and chiasm. Rddiology 66: 518-528, 1956. 7. Miller, N.R., Iliff, W.J., and Green, W.R.: Evaluation and management of gliomas of the anterior visual pathways. Brain 97: 743-754, 1974. March 1981 P.lrker, Smith, Reyes, Vuksanovic 8. Anderson, D.R., and Spencer, W.H.: Ultrastructural .lnd histochemical observations of optic nerve gliomas. Arch. Ophthd1m01. 83: 324-335, 1970. 9. Wong, I.G., and Lubow, M.: Management of optic glioma of childhood: A review of 42 cases. In Neuro­vphthdlmology Symposium of the University of Mi.lmi dnd the Bdscom Palmer Eye Institute, Vol. 6. C.V. Mosby Co.. St. Louis, 1972, chap. 6, pp.51-60. 10. Hoyt, W.F., MC?shel, l.G., Lessell, S., Schatz, N.J" and Suckling, R.D.: Malignant optic glioma in adult­hood. Brdin 96: 121-132,1973. 11. Harkin, J.c., and RC?ed, R. J.: Tumors of the periph­eral nervous system. In Atlds of Tumor Pathology, fase. 3. Armed Forces Institute of Pathology, Wash­ington, D.C., 1969. 12. Harper, CG., and Stewart-Wynne, E.G.: Malignant optic gliomas in adults. Arch. NeuroJ. 35: 731-735, 1978. 13. Oxenhandler, D.C and Sayers, M.P.: The dilemma of childhood optic gliomas. j. Neurosurg. 48: 34-41, 1978. 14. Lloyd, l.A.: Gliomas of the optic nerve and chiasm in childhood. Trans. Am. Ophtha/mol. Soc. 71: 488­535,1973. Acknowledgments The authors are grateful to Dr. Richard E. Brown for referring the patient and for providing extensive clinical follow-up information. They are also indebted to Dr. Walker B. Sorrell who performed the autopsy with a very careful dissection of the eyes and brain and kindly prOVided the tissues for pathologic review. Write For reprints to: J. Lawton Smith, M.D., P.O. Box 016880, Miami, Florida 33101. 43