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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 34  |  Issue : 2  |  Page : 161-163

Recurrent sphenoid wing meningioma extending into the orbit


1 Department of Ophthalmology, Amala Institute of Medical Sciences, Thrissur, Kerala, India
2 Department of Neurosurgery, Amala Institute of Medical Sciences, Thrissur, Kerala, India
3 Department of Plastic Surgery, Amala Institute of Medical Sciences, Thrissur, Kerala, India

Date of Submission31-Jan-2021
Date of Acceptance23-Mar-2021
Date of Web Publication30-Aug-2022

Correspondence Address:
Dr. Cherungottil Viswanathannair Radhadevi
Department of Ophthalmology, Amala Institute of Medical Sciences, Amala Nagar, Thrissur - 680 555, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/kjo.kjo_30_21

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  Abstract 


Sphenoid wing meningiomas are slow-growing tumors that originate from outer arachnoid meningeal epithelial cells. They can present with progressive symptoms of an orbital or temporal fossa mass, including temporal fullness, proptosis, globe displacement, ptosis, and impaired ocular motility. The ophthalmic manifestations of sphenoid wing meningiomas vary depending on the location of the primary tumor. In this case report, we discuss a 52-year-old female with meningioma of unusual presentation which was reported to be removed completely, and recurrence was noticed after a lapse of 3 years with subsequent extension into the orbit resulting in the structural and functional destruction of the eye.

Keywords: Arachnoid, meningeal epithelial cells, meningioma, proptosis, sphenoid wing


How to cite this article:
Radhadevi CV, Bhavadasan K, Jayakrishnan K. Recurrent sphenoid wing meningioma extending into the orbit. Kerala J Ophthalmol 2022;34:161-3

How to cite this URL:
Radhadevi CV, Bhavadasan K, Jayakrishnan K. Recurrent sphenoid wing meningioma extending into the orbit. Kerala J Ophthalmol [serial online] 2022 [cited 2022 Dec 4];34:161-3. Available from: http://www.kjophthal.com/text.asp?2022/34/2/161/355031




  Introduction Top


Meningiomas are typically benign slow-growing tumors that can be followed clinically and radiographically for recurrences. Sphenoid wing meningiomas are slow-growing tumors that originate from outer arachnoid meningeal epithelial cells.[1] The ophthalmic manifestations of sphenoid wing meningiomas vary depending on the location of the primary tumor.[2] The tumor can extend from the intracranial space into the orbit through bone, the superior orbital fissure, or the cavernous sinus. They can present with progressive symptoms of an orbital or temporal fossa mass, including temporal fullness, proptosis, globe displacement, ptosis, and impaired ocular motility. Tumors near the sella or optic nerve can cause visual field defects, optic disc edema, or atrophy. Eyelid edema and chemosis are also commonly encountered.[3] The average age of onset is 50 years with a higher incidence in women and Caucasians.[2] In this case report, we present a 52-year-old female with recurrent sphenoid meningioma extending into the orbit.


  Case Report Top


A 52-year-old female with a complaint of protrusion of the left eye (OS) for 3 years was referred to the ophthalmic outpatient department. History revealed proptosis, defective vision, and hearing on the left side initially in 2010. As per records, computerized tomogram and magnetic resonance imaging (MRI) confirmed left lateral sphenoid wing meningioma. Left pterional craniotomy with Simpson II excision of meningioma was done in June 2010 elsewhere. Histopathological findings showed WHO Grade I - Transitional meningioma. Postoperatively, the vision was 6/6 OS and the hearing was normal. After 3 years, she was again admitted for complaints of proptosis OS with restriction of eye movements. Vision OS was reduced to 6/60 Snellen. MRI showed a lesion in the extra- and intraconal compartments of the left orbit. She did not go for further follow-up. After a period of 2 years, she developed seizures with weakness of the right upper limb for which she was admitted and treated at a local hospital and later referred to our center. On examination, the right eye (OD) was normal; the left eyeball was atrophic which was pushed medially and downward by a large swelling that retracted the lids. The conjunctiva was stretched by the protruded swelling from the orbit and xerosed with irregular areas of crusting. The cornea was dry, opaque, and covered with crusts in the periphery [Figure 1]a. OS vision was no perception of light and MRI showed large soft tissue mass left orbit displacing the eyeball [Figure 2a and [Figure 2]b. She had associated difficulty in the left nostril due to thick mucoid discharge. OD was normal. Left frontotemporal craniotomy and excision of the tumor in the brain and modified exenteration left orbit were done and remaining flaps of upper and lower lids were used to cover the exenterated left orbit [Figure 1]b. Histopathological findings showed the meningothelial area with psammoma body [Figure 3]a and [Figure 3]b which further confirmed the diagnosis. Postoperatively, she had seizures which were controlled medically and right hemiparesis improved slowly with physiotherapy. The patient had a follow-up after 3 months which was uneventful. She had been referred to the Department of Radiation Oncology for further management.
Figure 1: (a) Cornea with dry, opaque, and covered with crusts in the periphery and (b) excised tumor

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Figure 2: (a and b) Magnetic resonance imaging showing left lateral sphenoid wing meningioma

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Figure 3: (a and b) Histopathology showing meningothelial area with psammoma body

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  Discussion Top


Meningiomas represent approximately 4% of all intraorbital tumors[2] and can arise from the optic nerve or extend into the orbit from adjacent structures. Meningiomas are generally benign and are the most common intracranial lesions, accounting for approximately 18% of all intracranial neoplasms. The WHO grade I being the most common, associated with prolonged, recurrence-free survival, but a subset is clinically more aggressive.[4] Secondary orbital meningiomas usually arise from the inner and outer aspects of the sphenoid wing and infiltrate medially into the orbit. They often compress the optic nerve by narrowing the optic canal. They may infiltrate the contents of the supraorbital fissure and cavernous sinus or compress the frontal and temporal lobes.[5] In this case, there was widening of the left optic canal and bony orbit with hyperostosis of the left lateral orbital plate, greater wing of the left sphenoid, and medial aspect of the left temporal bone.

These secondary tumors invariably progress and may grow to overwhelming size with time[5] as evident in this case. Here, the meningioma had recurred after a lapse of 3 years and has extended into the temporal cranial fossa, maxillary and ethmoid sinuses. Meningioma may recur, either because of incomplete removal or a true recurrence. The overall recurrence rates range from 13% to 40%.[6] The patient in this case report had complete removal as per records and thus can be considered as true recurrence.

The most important factor in the recurrence of meningiomas is the extent of removal. Complete removal of a meningioma is not always feasible and remnants of the tumor may be left behind in the dura, involved bone, venous sinus wall, or parts of the tumor adherent to vital structures thus leading to recurrence.[7] Secondary intraorbital meningiomas arise intracranially and subsequently extend into the orbit. These tumors can present as secondary in additional sites such as clinoid, planum sphenoidale, frontoparietal area, and olfactory groove. Some secondary tumors are quite large and both orbits can be involved.[4] Thus, it appears that meningiomas with orbital involvement, progress, leading to potential neurological compromise.[5] Our patient had developed seizures with weakness of the right upper limb.

A select group of lateral sphenoid wing meningiomas with tumor extension in the lateral or superolateral compartments of the orbital cavity may be successfully approached and removed through a lateral orbitotomy with resection of the sphenoid wing and without craniotomy. Cases with tumor extension to the anterior clinoid process and superior orbital fissure and those with extension medial to the axis of the optic nerve require a transcranial approach.[8] In this patient, both transcranial and orbital approaches were required. Radiation therapy has also been shown to be effective in controlling local tumor growth. Additional resection is another option. Complete resection is still the most desired result, as it may be curative. Current advances in peri and operative management, especially preoperative endovascular embolization, intraoperative computer-assisted image guidance, and refined surgical methods assist the surgeon in achieving optimum outcomes.


  Conclusion Top


This case report concluded as the meningioma was initially removed completely and recurrence was noticed after a lapse of 3 years with subsequent extension into the orbit with structural and functional destruction of the eye. Hence, the interventions should be done as early as possible in order to avoid both structural and functional damages in case of recurrent meningioma. Successful treatment of such lesions requires the expertise of ophthalmic and neurologic surgeons as teamwork.

Acknowledgment

The authors gratefully acknowledge the valuable help of Dr. Ajith TA, Professor, Department of Biochemistry, Amala Institute of Medical Sciences, Thrissur, Kerala, India, during the preparation of the manuscript.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, Burger PC, Jouvet A, et al. The 2007 WHO classification of tumours of the central nervous system. Acta Neuropathol.2007;114:97-109.  Back to cited text no. 1
    
2.
Call CB, Carter KD, Allen R. Meningiomas. Eye Rounds.org. August 30, 2011. Available from: http://EyeRounds.org/cases/137-meningiomas.htm. [Last accessed on 2013 Oct].  Back to cited text no. 2
    
3.
Holds JB, Chang WJ, Darairaj VD, Foster JA, Gausan RE, Harrison AR, et al. Basic Clinical Science Series. Orbit, Eyelids and Lacrimal System2011-2012. Am Acad Ophthalmol. Canada, 2017; p. 107-16.  Back to cited text no. 3
    
4.
Jain D, Ebrahimi KB, Miller NR, EberhartCG. Intraorbitalmeningiomas: a pathologic review using current World Health Organization criteria. Arch Pathol Lab Med.2010;134:766-70.  Back to cited text no. 4
    
5.
Boulos PT, Dumont AS, Mandell JW, Jane JA Sr. Meningiomas of the orbit: contemporary considerations.Neurosurg Focus 2001;10:E5.  Back to cited text no. 5
    
6.
Rockhill J, Mrugala M, Chamberlain M Intracranial meningiomas: an overview of diagnosis and treatment. C. Neurosurg Focus 2007;23:E1.  Back to cited text no. 6
    
7.
Zachariah SB, Khoromi S, Meningioma sphenoid wing. Disponibleen. Available from: htpp://emedicine.medscape.com/article/1215752. [Last accessed on 2010 Jun].  Back to cited text no. 7
    
8.
Mariniello G, Maiuri F, de Divitiis E, Bonavolontà G, Tranfa F, Iuliano A, et al. Lateralorbitotomy for removal of sphenoid wing meningiomas invading the orbit.Neurosurg.2010;66:287-92.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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