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Show ]. Gin. Neuro-ophthalmol. 3: 119-122, 1983. Multiple Meningiomas Involving the Orbit and the Cranial Cavity A Case Report ZAFER KARS, M.D, FIKRET ERGDNGbR, M.D. bZDEMIR GDR~AY, M.D. BEHSAN bNOl, M.D. Abstract Multiple meningiomas are reported in a case involving the orbit and cranial cavity. Meningiomas constitute approximately 3% of expanding lesions of the orbit, according to Reese6 Karp et al. 4 report an incidence of 5-10%. The term "multiple meningiomas" is applied to a condition in which a patient has more than one meningioma and is not simply a diffuse spread of one such neoplasm. J The incidence of multiplicity in meningiomas is 1-2%. i,3. 8. 9 In the article by Karp et aI., 4 the occurrence of intracranial meningioma, one with clinical diagnosis, in two patients with primary orbital meningiomas is reported. We present a patient with primary orbital meningioma from whom multiple cranial meningiomas were excised after an interval of 8 years. The patient had no manifestation of von Recklinghausen's disease. We have not encountered a report of primary orbital meningioma associated with multiple cranial meningiomas. Case Report A female patient, aged 16, was admitted to Hacettepe University Hospital on February 2, 1971, due to progressive right-sided proptosis with severe visual impairment. Past medical history revealed that a previous biopsy of the right orbital tumor, performed in an ophthalmology clinic on January 7, 1969, led to a diagnosis of meningioma. Neuro-ophthalmologic examination revealed right-sided proptosis of 11 mm, optic atrophy, and complete visual loss. Right carotid angiography June 1983 showed a peribulbar tumor stain. The orbit was explored through a transcranial approach and subtotal excision of the tumor was performed. The hi~tological diagnosis was meningioma. The patient was readmitted on February 24, 1972, because of recurring exophthalmos on the right. Examination revealed severe proptosis of 12 mm with total ophthalmoplegia. After a diagnosis of tumor recurrence, the eyeball was enucleated and orbital tissues were evacuated leaving only the periosteum and conjunctiva intact. Histological diagnosis was meningothelial and psammomatous meningioma. The tumor had encased the optic nerve (Fig. 1) and had also infiltrated the sclera. The patient was admitted for the third time on February 27, 1973, with an enlarging mass in the right orbit. local examination revealed edema of the eyelids and conjunctiva, increase in volume of the orbital tissues, and a hard mass palpable at the lateral margin of the orbit. Radioisotope scanning of the orbits showed a large isotope uptake on the right (Fig. 2). A transconjunctival orbitotomy and near total resection was performed and histological diagnosis was meningothelial meningioma. To avoid further recurrence, a total dose of 6,000 rads of radiotherapy was administered postoperatively from two portals-one aimed anteroposteriorly and the other lateromedially. The patient remained symptom-free for the following 7 years. She was admitted again on October 20, 1981, with a complaint of right-sided focal motor seizures of l-year's duration. Neurological examination revealed slight right hemiparesis with increased tendon reflexes on the same side. There was no sign of tumor recurrence in the right orbit. Radioisotope brain scan showed a large isotope uptake at the left parietal parasagittal area. Left carotid angiography revealed a large parietal parasagittal mass with diffuse staining (Fig. 3). Craniotomy performed on November 13, 1981, revealed two distant meningiomas with separate dural attachments. A parasagittal meningioma of about 7 cm diameter was accompanied by a more laterally 119 Multiple Meningiomas Figure 1. Photomicrogr~ph of the enucle~tion m~terial. Cross sections of optic nerve, optic nerve she~th, ~nd orbit~1 meningothelial meningiom~ (left to right) are demonstrated. (H&E, XIOO.) Figure 2. Orbital scan (Technetium"""' pertechnetate) in recurrent orbital meningioma. Increased uptake at the right is evident. placed convexity meningioma of about 2 cm diameter. The former was adherent to the falx and had infiltrated the superior longitudinal sinus. The latter with a separate dural attachment was isolated from the parasagittal one by a bridge of cerebral tissue. Both tumors were excised totally. Histologically, the parasagittal tumor was a fibroblastic meningioma. Histological diagnosis of the convexity tumor was meningothelial and psammomatous meningioma. 120 Figure .s. lett caroltd anglogr~mshowing diffuse staining of the p.lriet~1 pJr~s~gittal meningioma. A burr hole performed during the previous transcrani~1 orbitotomy is ~Iso visible. Discussion Primary orbital meningiomas may originate from the arachnoid around the optic canal, from the arachnoid around the posterior pole of the globe, or from ectopic foci located in the dura, in the periosteum and in the orbit.6 In the case presented here, the origin of the orbital meningioma was probably the arachnoid around the posterior pole of the eyeball. Journal of Clinical Neuro-ophthalmology The orbital neoplasm of this patient meets all the clinical and histological criteria laid out for a primary intraorbital meningioma by Karp et al. 4 A "young female" with a mass in the "right" orbit presents herself with "loss of vision" and "progressive exophthalmos." The tumor turns out to be a "meningothelial meningioma." Consecutive recurrences controlled finally by high-dose radiotherapy recalls its "aggressivity." Karp et al. 4 state that whether or not the meningiomas of the olfactory groove in their two cases were second tumors unrelated to the initial tumor (intraorbital meningioma) is speculative. Contrarily, in our case, it is almost sure that the orbital and cranial meningiomas are completely unrelated entities. The localization of the tumors precludes the possibility of direct relationship (Fig. 4). We think that the histological resemblance between the orbital meningioma and one of the cranial meningiomas is incidental. Frazer et al.~ report the occurrence of a suprasellar epidermoid cyst and a frontal falx meningioma in a patient with orbital fibrosarcoma. The patient had been irradiated for a supposed intracranial recurrence of the orbital tumor. The interval between the irradiation and the occurrence of the intracranial tumors was 33 and 35 years, respectively. The authors relate the intracranial neoplasia to the high-dose radiotherapy. figure 4. Illustration showing anatomical sites of the multiple meningiomas in the patient presented. lune 1983 Kars, Ergungor, Giir~ay, Onol In the case presented here, radiation cannot be condemned as the possible etiological factor in the development of intracranial neoplasia, since one of the beams was aimed through the right orbitotemporal region toward the ethmoid cells and left orbit, and the other through the right orbit toward the right sphenoid ridge and temporal lobe. Multiple meningiomas encountered in the left parietal area were distant from the irradiated tissues. The etiology of multiplicity in meningiomas is discussed by Levin et al.r. and Waga et al. 1Cl Levin et al. 5 offer origin from multicentric foci, spread along cerebrospinal fluid pathways and venous transmission as possible explanations. Waga et al. lO opposing these proposals, state that except for occurences related to von Recklinghausen's disease, multiple meningiomas develop on the basis of multicentric foci influenced by unknown tumorproducing factors. We believe that the patient presented here supports the hypothesis of multicentric foci. Cases of multiple meningiomas involving separate anatomical compartments (supratentorial, infratentorial, spinal) of the central nervous system have been reported by Sedzimir et al. 7 and Zervas et aLII The patient presented here is a peculiar case that adds the orbit to this list of anatomical sites which may be affected by multiple meningiomas. The authors conclude that multicentric foci, proposed as a possible etiological factor in the development of multiple meningiomas (in addition to other anatomical compartments of the central nervous system) may also involve the orbit. References 1. Cushing, H., and Eisenhardt, L.: Meningiomas: Their Classification, Regional Behaviour, Life History, and Surgical End Results. Charles C Thomas, Springfield, Ill., 1938, p. 785. 2. Frazer, AX., and Victoratos, G.C: Multiple disparate cranial neoplasms. Cancer 44: 2312-2315, 1979. 3. Frazier, CH., and Alpers, B.l.: Meningeal fibroblastomas of the cerebrum: A clinicopathologic analysis of seventy-five cases. Arch. Neural. Psychiatry 29: 935-989, 1933. 4. Karp, L.A., Zimmerman L.E., Borit, A., and Spencer, W.: Primary intraorbital meningiomas. Arch. Ophthalmol. 91: 24-28, 1974. 5. Levin, P., Gross, S. W., Malis, L.l., Kirshenbaum, A.H., and Hollin, S.A.: Multiple intracranial meningiomas. Surg. GynecoJ. Obstet. 119: 1085-1090, 1964. 6. Reese, A.B.: Expanding lesions of the orbit (Bowman lecture). Trans. Ophthalmol. Soc. u.K. 91: 85-104, 1971. 7. Sedzimir, CB., Frazer, A.K., and Roberts, 1.R.: Cranial ,lnd spinal meningiomas in a pair of identical twins. f. Neural. Neurosurg. Psychiatry 36: 368-376, 1973. 8. Taren, 1.A.: Meningiomas. In E.A. Kahn, Correlative Neurosurgery (2nd ed.), E.C Crosby, R.C Schnei- 121 Multiple Meningiomas der, and J,A. Taren, Eds. Charles C Thomas, Springfield, III. , 1969, pp. 68-86. 9. Vestergaard, E.: Multiple intracranial meningiomas. Acta Psychiatr. Neural. (Kbh.) 19: 389-411, 1944. 10. Waga, 5., Matsuda, M., Handa, H., Matsushima, M., and Ando, K.: Multiple meningiomas. Report of four cases. /. Neurasurg. 37: 348-351, 1972. 11. Zervas, NT., Shintai, A., KaJlar, B., and Berry, KG.: 122 Multiple meningiomas occupying separate neuraxial compartments. Case report. /. Neurasurg. 33: 216220, 1970. Write for reprints to: Ozdemir Gi.ir~ay, M.D., Department of Neurosurgery, Hacellepe University Faculty of Medicine, Ankara, Turkey. Journal of Clinical Neuro-ophthalmology |
