ABSTRACT Meningiomas are commonly encountered as intracranial brain tumours, but extracranial meningiomas do occur although seen rarely. Here we present a case of extracranial meningioma presenting as a mass over the medial canthus of left eye and the glabella with extension into the left ethmoid sinuses, without any neurological symptoms or signs. The patient underwent surgical excision, plastic surgical reconstruction and adjuvant radiotherapy after 3-dimensional conformal treatment planning

Ali, Nasir

Enam, Syed Athar

Ikram, Mubasher

Jangda, Abdul Qadir

Karsan, Farrok

Khan, Tahir Shafi

eCommons@AKU

eCommons@AKUSection of Otolaryngology, Head & Neck Surgery Department of SurgeryMay 2011Extracranial Meningioma: an Unusual Presentationof a Mass Over Inner Canthus of Left EyeNasir AliAga Khan UniversityMubasher IkramAga Khan UniversityTahir Shafi KhanAga Khan UniversitySyed Athar EnamAga Khan UniversityAbdul Qadir JangdaAga Khan UniversitySee next page for additional authorsFollow this and additional works at: http://ecommons.aku.edu/pakistan_fhs_mc_surg_otolaryngol_head_neckPart of the Otolaryngology Commons, and the Radiology CommonsRecommended CitationAli, N., Ikram, M., Khan, T. S., Enam, S. A., Jangda, A. Q., Karsan, F. (2011). Extracranial Meningioma: an Unusual Presentation of aMass Over Inner Canthus of Left Eye. Journal of The College of Physicians and Surgeons Pakistan, 21(5), 309-10.Available at: http://ecommons.aku.edu/pakistan_fhs_mc_surg_otolaryngol_head_neck/51AuthorsNasir Ali, Mubasher Ikram, Tahir Shafi Khan, Syed Athar Enam, Abdul Qadir Jangda, and Farrok KarsanThis article is available at eCommons@AKU: http://ecommons.aku.edu/pakistan_fhs_mc_surg_otolaryngol_head_neck/51Journal of the College of Physicians and Surgeons Pakistan  2011, Vol. 21 (5): 309-310 309INTRODUCTIONMeningioma is a well recognized tumour of the centralnervous system. Its incidence as intracranial diseaseis approximately 15-20%, but the primary extracranialmeningioma is exceedingly rare, accounting for only 2%or less which can extend into the paranasal sinuses.1Intracranial meningiomas arise from arachnoidmeninges of the brain, present with neurologicalsymptoms and signs, and typical locations include thecerebral convexities, falx cerebri, tentorium cerebelli,cerebellopontine angel and sphenoid ridge. Intracranialmeningiomas are not strictly brain tumours, since theyarise from arachnoid cap cells that form the externalmembranes of the brain. However, since they growinside the intracranial cavity and are followed byneurological symptoms and signs, they are usuallyclassified as brain tumours. Grossly meningiomas arewell circumscribed, firm, tan or grayish lesions;hyperostosis of adjacent bone may be present.2 Theyusually occur at an average age of 40-60 years. Unusual extracranial locations are on forehead, neckand forearm etc. We report an interesting case of anextracranial meningioma which presented as a swellingover the medial canthus of left eye, left supra orbitalridge and over bridge of nose also causing proptosiswithout any neurological symptoms and signs.CASE REPORTA 60 years old man presented with swelling on the leftperiorbital region for 2 years. During the previous oneyear he was operated twice outside our centre. This wasa second recurrence when he presented. Clinically thiswas a hyperemic mass at the medial canthus of left eyeand extending over the glabella (Figure 1). CT scan ofhead and neck showed a lesion involving predominantlythe left side of the nose measuring approximately 7 x 5 cmextending into the left orbit causing destruction andremodeling of the lamina papyracea and into the frontaland ethmoid sinuses on left side. Defect was alsoidentified in the fovea ethmoidalis with a possibleintracranial extension on the left side. It was biopsiedand histopathology was consistent with meningioma,grade I, according to WHO classification of CNSneoplasms. A panel of immunohistochemical stainingwas performed to confirm the diagnosis, cytokeratin(AE1/AE3), S 100, HMB 45 was negative and EMA waspositive. MRI brain ruled out any intracranial connection,however, there was proptosis of the left eye withinvolvement of the left medial rectus muscle. A team ofsurgeons comprising of ENT, neurosurgery and plasticsurgery operated on him. He underwent wide localexcision and plastic surgical reconstruction, withacceptably good cosmesis.Histological examination including immunohisto-chemistryconfirmed the diagnosis of meningioma. The frozensection examination showed close margins on medialcanthus of eye and scalp and the frontal and ethmoidsinuses were involved.On account of high risk of local recurrence he wasoffered adjuvant radiotherapy after 3-dimensionalconformal radiation treatment planning (Figure 2) toreceive 45 Gy dose @ 1.80 Gy/ fraction on 6 megavoltage energy on linear accelerator, followed by a boostof 09 Gy over five fractions to the areas of positivemargins (the medial canthus of left eye and frontal andethmoid sinuses) to a total radiation dose of 54 Gy. Hecompleted scheduled treatment in September 2008 andis free of local recurrence at 2 years follow-up.ABSTRACTMeningiomas are commonly encountered as intracranial brain tumours, but extracranial meningiomas do occur althoughseen rarely. Here we present a case of extracranial meningioma presenting as a mass over the medial canthus of left eyeand the glabella with extension into the left ethmoid sinuses, without any neurological symptoms or signs. The patientunderwent surgical excision, plastic surgical reconstruction and adjuvant radiotherapy after 3-dimensional conformaltreatment planning.Key words: Extracranial meningioma.   Ethmoid sinus meningioma.   Orbital meningioma.   Medial canthus mass. Department of Radiation Oncology1/Surgery2/Neurosurgery3,The Aga Khan University Hospital, Karachi.Correspondence: Dr. Nasir Ali, A-5, Al-Mustafa Square,Block 14, Gulshan-e-Iqbal, Karachi. E-mail: nasir.ali@aku.eduReceived  January 21, 2009;  accepted  March 12, 2011.Extracranial Meningioma: an Unusual Presentation of a MassOver Inner Canthus of Left EyeNasir Ali1, Mubasher Ikram2, Tahir Shafi Khan2, Syed Athar Enam3, Abdul Qadir Jangda1 and Farrok Karsan1CASE REPORTDISCUSSIONIntracranial meningiomas are the most common adultbenign intracranial neoplasms, whereas extracranialmeningiomas are rare tumours comprising 1-2% of allmeningiomas. The morphological features of extracranialmeningiomas are similar to intracranial counterparts.3The prognosis of meningiomas is generally favourable.However, in rare cases meningiomas are aggressiveand can occur as a malignant meningioma. Arndt et al.described a case of a papillary meningioma as anaggressive histological variant, which accounts for1.0-2.5% of all meningiomas.4 Extracranial sinonasaltract meningiomas often demonstrate an erosion of thesinus wall, with extension to the surrounding soft tissue,to the orbit and occasionally to the skull base5, as wasseen in this patient who presented with a soft tissuemass on the medial canthus of left orbit. Complete surgical excision of sinonasal meningioma isthe treatment of choice without the need for adjuvanttreatment.6 Relapses are possible in case of incompleteremoval of the primary lesion; this patient hadundergone excision twice and presented to us withsecond recurrence. The prognosis of extracranialmeningioma is always good if the excision is complete.3The CT scan and MRI are the investigations of choice tosee any intracranial connection or origin of the tumour,7and the differential diagnosis of extracranial meningiomaslocated in the nasal sinus region can include mucocele,sinonasal carcinoma and hemangioma.8The treatment of choice is the surgical resection andadjuvant treatment is reserved for incomplete excisionsand the positive margins. In the case being presented,patient was offered adjuvant radiation therapy becauseit was a recurrent disease and extending to frontal andethmoid sinuses and surgical pathology report showedclose margins at two sites.REFERENCES1. Jovanovic MB, Berisavac I, Perovic JV, Grubor A, Milenkovic S.Huge extracranial asymptomatic frontal invasive meningioma: acase report. Eur Arch Otorhinolaryngol 2006; 263:223-7. Epub 2005Jul 14.2. Perez CA, Brady LW, editors. Principles and practice of radiationoncology. 4th ed. Philadelphia: Lippincott Williams & Wilkins; 2003.  3. Murata H, Takahashi M, Takagi T, Katagiri H, Ito I, Ishida T.A case of primary extracranial meningioma of the forearam withbone invasion. Skeletal Radiol 2007; 36:551-3. Epub 2007 Mar 7.4. Arndt S, Wiech T, Mader I, Aschendorff A, Maier W. Rareextracranial localization of primary intracranial neoplasm.Diagn Pathol 2008; 3:14.5. Kainuma K, Takumi Y, Uehara T, Usami S. Meningioma of theparanasal sinus: a case report. Auris Nasus Larynx 2007; 34:397-400. Epub 2007 Feb 7.6. Gökduman CA, Iplikcioglu AC, Kuzdere M, Bek S, Cosar M.Primary meningioma of the paranasal sinus. J Clin Neurosci 2005;12:832-4.7. Saloner D, Uzelac A, Hetts S, Martin A, Dillon W. Modernmeningioma imaging techniques. J Neurooncol 2010; 99:333-40.Epub 2010 Sep 1.8. Thompson LD, Gyure KA. Extracranial sinonasal tractmeningiomas: a clinicopathologic study of 30 cases with areview of the literature. Am J Surg Pathol 2000; 24:640-50.310 Journal of the College of Physicians and Surgeons Pakistan  2011, Vol. 21 (5): 309-310Nasir Ali, Mubasher Ikram, Tahir Shafi Khan, Syed Athar Enam, Abdul Qadir Jangda and Farrok KarsanFigure 1: Hyperemic mass atmedial canthus of left eye and overglabella, pre-operative photograph.Figure 2: Radiotherapy 3-D CTplanning photogdraph showing isodelines covering planning treatmentvolume.l l l l lOl l l l l

Extracranial Meningioma: an Unusual Presentation of a Mass Over Inner Canthus of Left Eye

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Extracranial Meningioma: an Unusual Presentation of a Mass Over Inner Canthus of Left Eye

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