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ECTOPIC SECONDARY PARANASAL SINUS MENINGIOMA WITH ORBITAL EXTENSION TATIANA ROSCA MD, PHD1, NIKOLAOS MARAGKOS MD2, TEODORA VLADESCU MD, PHD1, GHERGHESCU GH. MD, PHD1 1Neuro-Surgery Department, Clinical Emergency Sf. Pantelimon Hospital, Bucharest, Romania Department, “Agios Panteleimonas” General Hospital, Pireas Greece The author responsible for manuscript preparation: Tatiana Rosca, 59 Gala Galaction Street, 011305 Bucharest, Romania, tel/fax: +40-21-6663751, E-mail: [email protected] 2Anaesthesiology Background. Description of a meningioma arising from the paranasal sinuses (bilateral frontal and ethmoidal sinus origin). Material and method. A 54-year-old patient with meningioma originating in bilateral frontal and ethmoidal sinus and invading the right orbit. Results. The management of the case is presented. Conclusion. Meningiomas originating in the paranasal sinuses are very rare. They are ectopic and there are only a few cases reported in literature. Keywords: ectopic meningioma, proptosis, paranasal sinusis INTRODUCTION Meningiomas account for 3 to 9% of all orbital tumors and are certainly not considered rare. [1] Primary orbital meningiomas represent between 0.4 to 2% of all intracranial meningiomas [1]. Secondary orbital meningiomas are considerably more common than their primary counterparts. The ectopic orbital meningiomas were first mentioned in the 80’s. [8,10]. BACKGROUND A 54-year-old man presented with an 11-month history of progressive protrusion and infradisplacement of the right eye and a 3-month history of horizontal diplopia (Fig. 1). MATERIAL AND METHOD On examination, the patient’s visual acuity was 20/20 OU, with normal visual fields and normal ocular fundi. Extraocular movements were full, but the patient had 5 mm of right proptosis and infradisplacement of the right globe. 32 CT scan revealed opacification of the right anterior ethmoid sinuses and both frontal sinuses (Figs. 2a-c). There was no evidence of any intracranial mass (2a-d). MRI revealed that the frontal sinus mass had destroyed the roof of the right orbit and was extending into the orbit, pushing the right eye downward (Fig. 3) The patient underwent a right superior anterior orbitotomy with resection of the mass from both the orbit and the sinuses. (Fig. 4). On histopathologic examination, the mass was found to be a meningioma that appeared to be arising from the paranasal sinus mucosa (Figs. 5a-c). RESULTS Postoperatively, the patient’s proptosis and diplopia resolved (Fig. 6), and CT scanning showed no significant residual tumor in either the orbit or paranasal sinuses. (Fig. 7) Romanian Neurosurgery Vol. XV nr. 1 ECTOPIC SECONDARY PARANASAL SINUS MENINGIOMA WITH ORBITAL EXTENSION FIG. 4 Surgery piece FIG. 1 Patient before surgery FIG. 2A FIG. 2B FIG. 2C FIG. 2D FIG. 5A FIG. 5B FIG. 5C FIG. 2A CT scan revealed opacification of the right anterior ethmoid sinus FIG. 2B CT-scan revealed opacification of both frontal sinuses FIG. 2C CT-scan revealed opacification of orbital region FIG. 2D CT-scan revealed no evidence of any intracranial mass FIG. 5a Paranasal sinus tumor infiltration (Obx10 H&E) FIG. 5b Spindle-shaped cells disposed in a whirlpool pattern (Obx10 H&E) FIG. 5c HE ob x 20 Celule meningoteliale in tesutul conjunctv al mucoasei sinusale FIG. 3 MRI revealed that the frontal sinus mass had destroyed the roof of the right orbit and was extending into the orbit FIG 6. Patient after surgery Romanian Neurosurgery Vol. XV nr. 1 33 TATIANA ROŞCA of Pathology. The location of these lesions included the nasal cavity (n = 14), the nasopharynx (n = 3), the frontal sinus (n = 2), the sphenoid sinus (n = 2), or a combination of the nasal cavity and ethmoid, frontal, sphenoid, and/or maxillary sinuses (n = 9). All these cases had been reported and treated by otolaryngologists [4-10]. REFERENCES FIG. 7 CT-scan showed no significant residual tumor in either the orbit or paranasal sinuses CONCLUSION Our case affected both the ethmoid and frontal sinus with orbital involvement, requiring a combined approach by an opththalmologist and neurosurgeon. DISCUSSION A meningioma outside the central nervous system (CNS) is considered to be ectopic [1]. Ectopic meningiomas are differentiated by their connection to the CNS (primary) or without a CNS connection (secondary) [2]. Meningiomas are benign tumors arising from the arachnoid cells that form the arachnoid villi and are generally seen in association with the dural sinuses. [3] Meningiomas originating in the paranasal sinuses are very rare. These tumors are thought to arise from embryonal arachnoid nests that were pinched off and left behind during embryonic development. Only a few such cases have ever been described. In 2000, Thompson and Gyure published 30 cases of sinonasal tract meningiomas diagnosed between 1970 and 1992 that had been retrieved from the files of the Otorhinolaryngic Registry of the Armed Forces Institute 34 1. Paul T. Boulos, M.D., Aaron S. Dumont, M.D., James W. Mandell, M.D., Ph.D., and John A. Jane, Sr., M.D., Ph.D., Meningiomas of the Orbit: Contemporary Considerations Neurosurg Focus 10(5), 2001 2. Kleihues P, Cavenee WK. WHO classification tumours of the central nervous system. Lyons, France: IACR, 2000: 176-184 3. Lang FF, Macdonald OK, Fuller GN, DeMonte F. Primary extradural meningiomas: a report on nine cases and review of literature from the era of computerized tomography scanning. J Neurosurg 2000;93:940-950. 4. Atherino CCT, Garcia R, Lopes LJ: Ectopic meningioma of the nose and paranasal sinuses (report of a case). J Laryngol Otol 99:1161-1166, 1985 5. De SK, Chatterjee AK, Misra AK: An unusual presentation of meningioma of the frontal sinus. J Laryngol Otol 100:711-714, 1986 6. Fagerlund M, Stenling R, Söderberg O: A subfrontal meningioma with primary origin from the nasal cavity. Acta Otolaryngol 95:365-370, 1983 7. Godel V, Samuel Y, Shanon E: Maxillary meningioma appearing as exophthalmos. Arch Otolaryngol 107:626-628, 1981 8. Ho KL: Primary meningioma of the nasal cavity and paranasal sinuses. Cancer 46:1442-1447, 1980 9. Kumar S, Dhingra PL, Gondal R: Ectopic meningioma of the paranasal sinuses. Childs Nerv Syst 9:483-484, 1993 10.Thompson LD, Gyure KA, Extracranial sinonasal tract meningiomas: a clinico- pathologic study of 30 cases with a review of the literature. Am J Surg Pathol. 2000 May; 24(5):64050. Romanian Neurosurgery Vol. XV nr. 1