Full Text Article Open Access

Citation: Zgolli HM, Mabrouk S, Yeadeas D, Zeghal I, Fekih O, Yeadeas M. Primary extradural ectopic orbital Meningioma. Jr.med.res.2021;4(1):10-12.

Zgolli et al © All rights are reserved. https://doi.org/10.32512/jmr.4.1.2021/10.12

Submit your manuscript: www.jmedicalresearch.com

Case report

Zgolli Hsouna Mehdi

1,3, *

, Mabrouk Sonya

1,3

, Yeadeas Dahmeni

2,3

, Zeghal Imen

1,3

, Fekih Olfa

1,3

, Yeadeas Monther

2,3

Primary extradural ectopic orbital Meningioma.

1: Department of Ophthalmology,

Hedi Raies Institute, Tunis, Tunisia

2: Department of ophthalmology,

Military Hospital, Tunis, Tunisia

3: College of medicine, Tunis, Tunisia

* Corresponding author

Correspondence to:

hsouna_zgolli@yahoo.com

Publication Data:

Submitted: January 02,2021

Accepted: April 12,2021

Online: May 30,2021

This article was subject to full

peer-review.

This is an open access article distributed

under the terms of the Creative Commons

Attribution Non-Commercial License 4.0

(CCBY-NC) allowing sharing and adapting.

Share: copy and redistribute the material in

any medium or format.

Adapt: remix, transform, and build upon the

licensed material.

The work provided must be properly cited

and cannot be used for commercial purpose.

Orbital meningioma is a rare benign tumor. However, the existence of ectopic orbital

meningiomas was debated for long time and this lesion might be underreported. Complete

surgical excision could achieve a good prognosis without visual impairment. The recurrence

rate is still considerable. The aim of this report was to highlight the diagnostic difficulties and

management characteristics.

Keywords

Orbit; meningioma; origin, diagnosis; management.

Introduction

Orbital meningioma is a rare tumor. This entity is more frequently diagnosed in adult

patients. Meningiomas that arise from the arachnoid layers of the optic nerve are

considered as primary. Secondary orbital meningioma is defined as an extension of an

intracranial process, usually from the sphenoidal wind [1]. Orbital meningioma may arise

apart from optic nerve and without any connection with the intracranial meninges. In

such cases the lesion is classified as ectopic orbital meningioma [2]. This entity is very

rare; few cases are reported in the literature. Its origin is still controversial. We herein,

report a case of an ectopic orbital meningioma with compressive optic neuropathy.

Observation

We report the case of a 37-year-old female presenting high myopia (-6.25 in both eyes).

She complained three months after childbirth of progressive protrusion of the right eye,

decreased visual acuity and amputation of the lower visual field. A swelling of the upper

eyelid appeared later. The examination of the left eye was normal. The examination of

the right eye diagnosed an axial and a non-reducible exophthalmos and a best corrected

visual acuity of only 2/10. Eye fundus showed papillary edema stage 2. Otherwise, the

physical examination was normal.

The Lancaster’s test was normal. The Goldman visual field test revealed enlargement of

the of the blind spot. Orbital tomography showed a right exophthalmos grade 2, with a

right retro-orbital mass of 2 cm. The lesion had cystic and tissular mixed pattern and

partially enhanced after contrast injection. MRI scan right intra-conical and retro orbital

mass of 22x20 with isoT1 and hyper T2 signals and heterogeneous enhancement after

gadolinium injection. A mass effect was noted on the right eye and optic nerve without

invasion (figure1).

Surgery was indicated and performed by multidisciplinary team including neurosurgeon

and ophthalmologist. A right sub frontal approach with orbital roof section was indicated

(figure2). The tumor was completely resected, and the specimen was sent to the

pathology department. Histological examination concluded to transitional meningioma

grade1. The postoperative course was marked by ophthalmoplegia and ptosis which

regressed completely after 3 months. The ophthalmological examination at 1 month

showed visual acuity of 6/10 and mild papillary edema. Increase of the visual acuity to

8/10 and normal fundus examination were seen later at 6 months. No recurrence of the

tumor was noted in The MRI scan done 2 years after the procedure.

Abstract

Primary extradural ectopic orbital Meningioma

Citation: Zgolli HM, Mabrouk S, Yeadeas D, Zeghal I, Fekih O, Yeadeas M. Primary extradural ectopic orbital Meningioma. Jr.med.res.2021;4(1): 10-12.

Zgolli et al © All rights are reserved.

Submit your manuscript: www.jmedicalresearch.com

Figure 1: Orbito-cerebral MRI : (a) T1 axial image: well limited mixed intra right retro-orbital tissular mass 22 x20 mm; iso intense T1.

(b,c): heterogeneous enhancement after gadolinium injection. (d): T2 axial image: hyperintense tissular mass signal.

We noted the independence of the mass which is only compressing the optic nerve.

Figure 2: Surgical site access and resected tumor features.

Primary extradural ectopic orbital Meningioma

Citation: Zgolli HM, Mabrouk S, Yeadeas D, Zeghal I, Fekih O, Yeadeas M. Primary extradural ectopic orbital Meningioma. Jr.med.res.2021;4(1): 10-12.

Submit your manuscript: www.jmedicalresearch.com

Discussion

Meningiomas are one of the most frequent neoplasms arising from the meninges. These tumors belong to the

arachnoid villi structures [3]. Commonly, meningiomas are attached to the dura and grow in the cranial cavity or

intraspinal region. However, several ectopic location such as mandibular or parapharyngeal have been described.

The orbital ectopic meningioma remained controversial, and less than 25 cases were reported till 2018 [4]. Primary

orbital meningioma accounted for 5-10% of all orbital neoplasms and mainly diagnosed in adult patients Primary

orbital meningiomas originate from the arachnoid layer of the optic nerve sheath. Approximately 70% of orbital

meningiomas are intracranial neoplasms originating at the sphenoid ridge, with orbital and intraluminal extensions

[5]. The debate about its origin evidence may be related to the paucity or incomplete character of the of the

published series. Congenital and traumatic causes have been evoked by some authors[6]. The preoperative

diagnosis is difficult because of the non-specificity of the symptoms. The magnetic resonance scan may contribute

to distinguish this tumor from the other neoplasms of the sphenoid wing and remain the standard for diagnostic

and follow up imaging [7]. Surgical excision is the gold standard of the management and must be complete to

avoid recurrence. The management of recurrent tumors is more challenging and may require external beam

radiotherapy (EBRT) with all its possible retinal side effects [8]. In this report we tried to demonstrate that orbital

meningioma may arise apart from optic nerve and without any connection with the intracranial meninges. This

could make reasonable to consider it as ectopic despite the confusing anatomical factors.

Conflict of Interest: None

References

[1] Satija CE, Mehta VJ, Maltry AC, Nussbaum E, Nussbaum L, Harrison AR. A report of intracranial meningioma recurring as ectopic orbital meningioma associated with

basal encephalocele. Ophthalmic Plast Reconstr Surg. 2020;36:e19-e21.

[2] Tendler I, Belinsky I, Abramson DH, Marr BP. Primary extradural ectopic orbital meningioma. Ophthalmic Plast Reconstr Surg. 2017;33:S99-S101.

[3] Tan LT, Stewart CM, Sheerin F, Mac Donald B, Silva P, Norris JH. Ectopic orbital meningioma: Fact or fiction? Orbit. 2017;36:144-46.

[4] Yeşiltaş YS, Gündüz K, Okçu Heper A, Erden E. Ectopic rhabdoid meningioma of the orbit in a child: case report and review of the literature. J Neurosurg Pediatr.

2018;22:151-57.

[5] Huang X, Tang D, Wu T, Jian T, Sun F. Ectopic orbital meningioma: a retrospective case series. BMC Ophthalmol. 2018;18:296.

[6] Gündüz K, Kurt RA, Erden E. Ectopic orbital meningioma: report of two cases and literature review. Surv Ophthalmol. 2014;59:643-8.

[7] Lee DH, Sim HS, Hwang JH, Kim KS, Lee SY. Extracranial Meningioma Presenting as an Eyebrow Mass. J Craniofac Surg. 2017;28:e305-e307.

[8] Luzzi S, Zoia C, Rampini AD, Elia A, Del Maestro M, Carnevale S,et al. Lateral transorbital neuroendoscopic approach for intraconal meningioma of the orbital apex:

Technical nuances and literature review. World Neurosurg. 2019;131:10-17.