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Show f. Clin. Neuro-ophthalmol. 3: 169-179, 1983. Managing Menacing Meningioma J. LAWTON SMITH, M.D. JOHN A. McCRARY, III, M.D. BRONSON S. RAY, M.D. MARIO M. VUKSANOVIC, M.D. Abstract The role of irradiation therapy in the management of meningiomas involving the anterior visual system is under active clinical investigation. This report documents the value of radiotherapy in a patient with a histologically verified psammomatous tuberculum sellar meningioma and presents a 15-year postsurgical follow-up. The patient experienced a return of vision from 11/200 to 20/60 in his only seeing eye following 5,000 rads to the intracanicular and intracranial portions of right optic nerve. He is able to practice his profession 3 years following irradiation therapy. The classification of optic nerve involvement by meningioma is discussed. The management of extensive, recurrent, and multiple meningiomata presents formidable clinical challenges requiring a careful and individualized approach in each instance. The value of a combined surgical and radiotherapeutic approach is seen in the following scenario which is documented because of surgical and histopathologic confirmation, a later dramatic response to radiotherapy, and a clinical follow-up for over 14 years. Case Report A 35-year-old, right-handed white married male attorney was first seen at the Bascom Palmer Institute by one of us (JAM) on September 8, 1969, complaining of decreasing vision in the right eye for I lh years. He enjoyed good vision in both eyes until 1950, when at age 18, he was struck on the left temple by a thrown bicycle seat, but was not rendered unconscious. One year later, he happened to cover his right eye, and found that his left eye was totally blind. He ignored that until he began to note difficulty with the remaining fight eye. Examination on September 8, 1969, revealed a corrected vision of 20/25-1 in the right eye, and no From the Bascom Palmer Eye Institute. Department of Ophthalmology, University of Miami School of Medicine, Miami, Florida. September 1983 light perception in the left eye. An exotropia of 20 diopters and a right hypertropia of 10 diopters were present. The left pupil was amaurotic. Both optic discs were pale, more so in the left eye. An upper temporal visual field defect was present in the right eye (Fig. 1). Olfaction and corneal sensation were decreased on the left. The impression was chiasmaI compression, with meningioma being first choice in the differential diagnosis. Arteriography and pneumoencephalography confirmed this interpretation, and craniotomy was performed September 16, 1969, by one of us (BSR). This revealed a tuberculum sellar meningioma which was removed as completely as possible, but the left optic nerve had been sacrificed by the tumor. Histopathologic examination of the surgical specimen revealed typical psammomatous meningioma (Figs. 2 and 3). A postoperative examination on October 7, 1969, revealed a corrected vision of 20/ 25-3 in the right eye, no light perception in the left eye, and marked improvement was noted in the field of the right eye (Fig. 4). The patient later dropped out of medical followup. He returned to this department 11 years later because of decreasing vision in the right eye. Examination on June 10, 1980, by one of us (JLS) revealed a corrected vision of 11/200 (eccentric in the upper nasal field) in the right eye, and the left eye had no light perception. Visual field examination revealed only a 10-15° upper nasal remnant of field in the right eye (Fig. 5). Primary optic atrophy was present in both eyes, and classic optociliary shunt collaterals were seen on the left disc (Figs. 6 and 7). The impression was recurrent meningioma, involving both optic nerves, producing .1 right third nerve palsy, with involvement of sphenoid ridge and cerebellopontine .1ngle on the right, and falx on the left. The patient was using a white cane for ambulation. Surgery was advised, but the patient absolutely and adamantlv refused repeat craniotomy. Accordingly, he was given a course of irradiation therapy by one of us (MV), with 5,000 rads being given to right intracanalicular and intracranial optic nerve region between June 12, 1980, and July 22, 1980. The subsequent course of improvement in visual acuity in the patient's only seeing eye is documented in Table 1. 169 Menacing Meningioma Opera tor --C:j am _ De te 9_-_7-_6_9 _ Test objects -~ VIIiOD ----I)~n,l.p!.._ LE. VISUAL. FIELDS (peripheral) (CeDtrai) R.E. 3/330 white (0.6) sc Test objects Al.mark per lrtreter PRE-OP FIELDS V11 lOll__2_0/ ...2_5_-_2_ Figure 1. Preoperative visual fields, September 7, 1969. Figure 2. Histopathology of surgical specimen from operation on September 16, 1969. Journal of Clinical Neuro-ophthalmology Smith, McCrary, Ray, Vuksanovic Figure 3. Note psammoma bodies (psammomatous meningioma). Opera tor ja!.Jl=- _ D. te 1_1-_5_-_6_9 _ Test object' _ VISUAL. FIELDS (per Iphra J) Test objects 3/330 wbite (0,6) sc Aimark perimeter LE. -JW> VI,loD _ (CeDtnt) R.Eo MlUO Craniotomy 9-16-69 Dr.Bronson Ray POST-OP FIELDS 20/20-3 VI.ioD _ Figure 4. Postoperative visual fields, 2'/2 weeks following surgery. Note improvement in visual field in right eye. September 1983 171 Menacing Meningioma jls Date 6-10-80 _ Operator _ Test objects _ VISUAL FIELDS (peripheral) T • bj 10/330 W (0) int sc e,. 0 ec: tLI--T:!'=~""'=:::"':"=""':-;::",A1mark per1mefer LE. R.E. Villon _ (Cantral) 11 years POST-OP --- NOW PRE-RADIOTHERAPY VlsioD 11/200 _ Figure 5. Visual fields, June 10, 1980 (11 years postoperative, but prior to irradiation therapy). Figure 6. Fundus photograph, right eye, June 19, 1980. Note optic atrophy. Journal of Clinical Neuro-ophthalmology Smith, McCrary, Ray, Vuksanovic Figure 7. Fundus photograph. left eye, June 19, ]980. Note optociliary shunts at 1:45 and 7:00 on this atrophic disc. The patient's visual field improvement in the right eye is seen immediately at the end of irradiation therapy (Fig. 8), 4 months later (Fig. 9), and 2 years out (Fig. 10). Reading vision improved from 10 M to 2.5 M print in the right eye. Examination on August 7, 1982, revealed a corrected vision of 20/60 in the right eye and no light perception in left eye. The field at that time is seen in Figure 10. External examination then is seen in Figures 11-16. These document the right III nerve palsy, show the ptosis crutch glass which helped keep his right eye open, and documented the interesting motility pattern. In essence, for practical purposes, the seeing right eye could not move, whereas the blind left eye could move (Figs. 1116). Computed tomography of orbits and brain was obtained November 26, 1982 (13 years after surgery and 21/2 years after irradiation therapy). Per- TABLE 1. Visual Acuity Response to Radiotherapy tinent findings are seen in Figures 17-21. Axial views of the orbits showed enlarged orbital optic nerves with notable calcification of the sheaths showing the typical railroad track or tramtrack sheath sign of optic nerve meningiomas. A coronal reconstruction of the orbits shows the enlarged calcified optic nerve meningiomas in Figure 18, and a midline intracranial recurrence is also evident in this figure. A lateral orbital reconstruction shows the meningioma enveloping this optic nerve and recurrence in the tuberculum-planum sphenoidale region intracranially (Fig. 19). Notable bilateral recurrence into the cavernous sinuses is seen on a coronal section (Fig. 20). Finally, recurrence in region of the falx anteriorly with extensive involvement of sphenoid ridge and tentorial margin on the left, and also right cerebellopontine angle, is seen in Figure 21. A telephone communication with the patient's wife in May 1983, disclosed that the vision was still holding in the patient's right eye, and he was continuing to practice his profession nearly 3 years following radiotherapy. Date 6-]0-80 6- ]2-80/7-22-80 6-26-80 7-24-80 11-]8-80 8-7-82 September 1983 Corrected Ri!:ht Eye Acuity 1]/200 Radiotherapy (5,000 rads total) 15/200 20/200 20/70 20/60 Comment This patient is of interest in documenting the following: 1) the presence of a histologically proven psammomatous tuberculum sellar meningioma removed at age 35 in 1969; 2) recurrence with vision dropping to 11/200 in his only eye, which responded to irradiation therapy and improved to 173 Menacing Meningioma Operator j Is D. te 7_-_2_4_-_8_0 _ r~st objecte _ VISUAL FIELDS (;perlpberal) Test objectl 10/330 W (0) int sc Almark perimeter 5000 Rads given 6-12-80 to 7-22-80 Visioll 20/200 (5 M) IMMEDIATELY AT END OF RADIOTHERAPY COURSE R.E. I,'isioll _ (Central) LEo Figure 8. Visual fields on July 24, 1980 (immediately at end of radiotherapy). )pera tor j :rn ,_rn_, _ Da te 11_-_1_8_-_8_0 _ ~est objects VISUAL FIELDS (;perlpberal) 10/330 white (0) int Test objects"-"11Srl"e~AHlt'1'iUl!':'a'l"l[rll\:~prl'eeiT:iimili!!e~L'I"!e'l"[- 4 MOS. AFTER RADIOTHERAPY Visioll _ LEo (C.ntral) Vision 20/70- (J 6) Figure 9. Visual fields on November 18. 1980 (4 months after radiotherapy). Journal of Clinical Neuro-ophthalmology Smith, McCr.uy, Ray, Vuksanovic -_.==================== Operator j Is . _ Date 8-7-82 _ :'est objects VI,loD L.E. VISUAL. FIEL.DS (peripheral) (C'Dual) JU. Tut objects 10/330 W (0) int sc Aimark perimeter TWO YEARS AFTER RADIOTHERAPY Vi 1I'0D 20/60 (2,5_ M) Figure 10. Visual fields on August 7. 1982 (2 years after radiotherapy) Compare with fields prior to radiotherapy (Fig. 5). Figure 11. External appearance of patient. August 7, 1982. 20/60; 3) presence of classic computed tomographic changes consistent with bilateral optic nerve sheath meningiomas; and 4) documented improvement in acuity and field over 2 years after radiotherapy in this patient. September 1983 Discussion Optic nerve meningiomas, formerly considered rare, are now found so often by late generation, high-resolution computed tomography as to constitute a rather ordinary problem in neuro-ophthalmologic practice. However, as the recognition of these tumors has become easier, to some extent the management has become more complicated. It will be evident that some of the comments to be made here can be applied to meningiomas in general. However, if we confine our remarks simply to meningiomas involving the optic nerve, there now appears to definitely be more than one entity among these patients. First of all, optic nerve meningiomas can be classified clinically into four types: 1) primary, 2) secondary, 3) recurrent, and 4) multiple. Dr. Richard Lindenberg l previously gave a diagrammatic representation of six possible ways that orbital meningiomas might arise. These included: 1. Intracranial tumor 2. Intracanalicular villus 3. Subdural arachnoid involvement 4. Intradural ectopia (which may grow in an exophytic or endophytic manner in the optic nerve sheath) 5. Periosteal ectopic arachnoid rest 6. Extradural ectopic arachnoid rest 175 Menacing Meningioma Figure 12. Note ptosis crutch on right eye glass. Figure 13. Patient fixing in primary position, August 7, 1082. Figure 14. Right g,lZl' Journal of Clinical Neuro-ophthalmology Smith, McCrary, Ray, Vuksanovic Figure 15. Up gaze. Figure 16. Down gaze. Note that the seeing right eye moves little, but blind left eye moves well. Since the intradural ectopia is so important from the surgical point of view, one might extend this classification as follows: 1. Intracranial tumor. Arachnoid cell rests are encountered commonly at the falciform fold of dura at intracranial end of optic canal. This appears to be a common site of origin for meningiomas" 2. Intracanalicular villus 3. Subdural arachnoid involvement, producing a sleeve or cuff meningioma 4. Intradural ectopic origin growing within the vaginal sheath (endophytic) 5. Intradural ectopic origin growing outside the vaginal sheath (exophytic) 6. Extradural ectopic arachnoid rest in the orbit 7. Periosteal ectopic arachnoid rest in the orbit It appears that an attempt to differentiate these different types of origin of optic nerve meningiomas within the orbital and intracanalicular portions is of more than academic interest. Therefore, September 1983 if the meningioma arises from ectopic arachnoid cell rests within the orbit, but outside the optic nerve sheath, and then grows to compress and invade the dural sheath, the possibility of removing such a lesion by microsurgery might be clinically feasible. However, if the meningioma arises within the sheath itself and intimately involves the small pial vessels of the optic nerve, any attempt to "peel off" or "shave off" such a meningioma would yield virtually a 100% chance of infarcting the optic nerve even in the best ot hands. It is not possible to differentiate all of these types of origin of optic nerve meningiomas at this time. However, this subject is now under active investigation and appears to hold promise in determining the management of these cases later. The case for irradiation therapy for primary optic nerve sheath meningiomas has been previously reported..' This particular patient obviously had much more extensive involvement than simply 177 Menacing Meningioma Figure 17. Axial computed tomographic scan. November 26,1982. Note bilateral optic nerve sheath meningiomas. Figure 18. Coronal scan, November 26, 1982. Note enlarged optic nerves. Figure 19. Llteral reconstruction-note thickened, calcified optic nerve sheath. Journal of Clinical Neuro-ophthalmology Smith, McCrary, Ray, Vuksanovic figure 20. Coronal scan-showing bilateral cavernous sinus involvement. Figure 21. Note extensive intracranial involvement in this patient. bilateral optic nerve sheath meningiomata. Therefore, there was involvement of tuberculum, planum, cavernous sinuses, angle, falx, and tentorial margin on the left in this patient. Because he absolutely refused surgical decompression, a course of radiotherapy was given to the right optic nerve. The patient had a definite clinical response which has held for 3 years to date. There appears to be little doubt that some meningiomas are radiosensitive to some extent, and that some of these patients can be visually palliated by this modality. Further evaluation of radiotherapy not only for primary optic nerve sheath meningiomas, but for extensive and recurrent and multiple intracranial meningiomas, particularly in patients who appear poor surgical risks, appears clinically warranted. References 1. Lindenberg, R. (cited by Walsh, F.B.): In Neuroophthalmology Symposium of the University of September 1983 Miami and the Bascom Palmer Eye Institute, J.L. Smith, Ed. Huffman Publishing Co., Hallandale, Florida, 1970, Chap. 18, p. 261. 2. Perez, G.M., Parker, J.C, Jr., and Smith, J.L Arachnoidal cap cell hyperplasia of the intracanalicular optic nerve sheath. ]. Clin. Neuro-ophthalmoJ. 1: 173-183,1981. 3. Smith, J,L., Vuksanovic, M.M., Yates, B.M., and Bienfang, D.C: Radiation therapy for primary optic nerve meningiomas. ]. Clin. Neuro-ophthalmoJ. 1: 85-99, 1981. Acknowledgments The authors thank Dr. James M. Mitchell who provided follow-up notes; and Dr. Robert M. Quencer, who reviewed the computerized tomographic scans on this patient. Write for reprints to: J. Lawton Smith, M.D., Bascom Palmer Eye Institute, P.o. Box 016880, Miami, Florida 33101. 179 |
