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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 1  |  Issue : 4  |  Page : 752-755

Transcleral local resection of an ancient intraocular schwannoma: A case report and recommendations on management


Department of Vitreoretina and Ocular Oncology, Sankara Eye Hospital, Bangalore, Karnataka, India

Date of Submission07-Dec-2020
Date of Acceptance24-May-2021
Date of Web Publication09-Oct-2021

Correspondence Address:
Dr. Preethi Sridharan
Department of Vitreoretina and Ocular Oncology, Sankara Eye Hospital, Varthur Road, Kundalahalli Gate, Munnekollal, Bangalore - 560 037, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_3600_20

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  Abstract 


A 24-year-old male presented to us with a slow growing, painless mass lesion in his left eye. A dome-shaped, well-defined, nontender, nodulocystic, immobile, transilluminant subscleral mass was seen in the nasal quadrant. Infiltration of ciliary body, iris, choroid, and sclera by a suprachoroidal mass of variable echogenicity was noted on B scan ultrasonography (USG). Incisional biopsy hinted toward intraocular schwanomma. Considering the infiltration seen on USG B scan, progressive increase in size, and relatively good prognosis if intervened early, a transcleral local resection was performed. Histopathology and immunohistochemistry confirmed an ancient schwannoma. Patient recovered without complications.

Keywords: Ancient schwannoma, intraocular schwannoma, local resection, transcleral resection


How to cite this article:
Shanmugam MP, Sridharan P, Ramanjulu R, Mishra DK. Transcleral local resection of an ancient intraocular schwannoma: A case report and recommendations on management. Indian J Ophthalmol Case Rep 2021;1:752-5

How to cite this URL:
Shanmugam MP, Sridharan P, Ramanjulu R, Mishra DK. Transcleral local resection of an ancient intraocular schwannoma: A case report and recommendations on management. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Oct 26];1:752-5. Available from: https://www.ijoreports.in/text.asp?2021/1/4/752/327684



Intraocular schwannoma is a rare tumor arising from the Schwann cells of the ciliary nerves, as they run through the uveal tract.[1] We present a rare case of an ancient ciliary body schwannoma, managed by transcleral local resection. To our knowledge, this is the only case of ancient intraocular schwannoma managed by local resection.


  Case Report Top


A 24-year-old male presented to us with a painless, slow-growing mass lesion in his left eye, first noticed by him 2 years ago. His best-corrected visual acuity (BCVA) was 20/20 in both eyes. Left eye had a well-defined, dome-shaped, nontender, nodulocystic, immobile, subscleral mass in the nasal quadrant measuring 9 mm × 7 mm, with an adjacent dilated episcleral vessel [Figure 1]a. The mass was brilliantly transilluminant. Rest of the anterior segment was normal. Fundus showed a mass effect in the superonasal periphery [Figure 1]b. Right eye was unremarkable. Intraocular pressure was 12 mmHg in both eyes.
Figure 1: Anterior segment photograph of the left eye showing a well-defined, dome-shaped, subscleral mass in the nasal quadrant measuring 8–9 mm × 7 mm, with an adjacent dilated episcleral vessel. Overlying sclera appears to have thinned out (a). Fundus photograph of the left eye showing a mass effect in the periphery of the superonasal quadrant (b). B scan ultrasonography showing a mass of variable echogenicity in the ciliary body region, with infiltration of the choroid, iris, and sclera. No orbital shadowing was seen (c).

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B scan ultrasonography showed a mass of variable echogenicity in the ciliary body region, with infiltration of the choroid, iris, and sclera, with no orbital shadowing [Figure 1]c. An ultrasound biomicroscopy done elsewhere revealed a mass lesion arising from the ciliary body with an extension to the iris. Magnetic resonance imaging orbit showed a well-defined lesion in the subconjunctival space, hypointense on T1, mixed on T2, with a cystic component.

As the evaluation was inconclusive of a diagnosis, we went ahead with an incisional biopsy [Video Clip 1]. Histopathological examination (HPE) predominantly showed spindle cells, scattered sinusoidal vessels, stroma with minimal myxoid and microcystic degeneration, suggestive of an Antoni B pattern. There was nuclear atypia, mitosis of less than 5 per 10 high power field (HPF) [Figure 2]. Immunohistochemistry revealed diffuse nuclear and cytoplasmic positivity for S-100, 15% Ki 67, negative for HMB 45, desmin, smooth muscle actin, and cytokeratin. Features were suggestive of an intraocular schwannoma.
Figure 2: Histopathological examination of incisional biopsy revealing predominantly spindle cells, scattered sinusoidal vessels, stroma with minimal myxoid and microcystic degeneration, suggestive of an Antoni B pattern. There is nuclear atypia and mitosis of less than 5 per 10 high power field

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A diagnosis of ciliary body schwannoma with suspected local extension was made. Under hypotensive general anesthesia, a transcleral local resection of the mass was performed [Video Clip 2]. 360° limbal peritomy was done and the recti were tagged. A full thickness, rectangular scleral flap was raised in the nasal quadrant. The tumor was noted to be suprauveal, predominantly over the ciliary body, with an extension onto the choroid [Figure 3]. A meticulous extracapsular dissection revealed that there was no infiltration into the surrounding tissues. Cryo-assisted extraction of the tumor was done and scleral flap was closed with interrupted monofilament nylon sutures.
Figure 3: Transcleral local resection: the tumor was noted to be suprauveal in location, encapsulated, with no infiltration into the surrounding tissues

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HPE showed a multinodular mass with a few cystic areas. The periphery showed melanophages. The cellular areas were composed of epitheloid cells with reniform nuclei, tiny nucleoli and pseudoinclusions, as well as spindle cells arranged in Antoni A pattern with verocay bodies and Antoni B pattern. Hypocellular areas had myxoid stroma with ectactic vessels and perivascular hyalinization. Areas of microcystoid degeneration were present. Nuclear atypia was seen. Mitosis was less than 4 per 10 HPF. Features were typical of an ancient schwannoma.

No intraoperative or postoperative complications were noted. Patient recovered well and retained his BCVA of 20/20 [Figure 4].
Figure 4: Anterior segment photograph of the left eye 2 weeks post local resection (a). Fundus photograph of the left eye showing chorioretinal atrophy in the periphery of superonasal quadrant 3 weeks post local resection (atrophy post cryotherapy). No other abnormality was noted (b)

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  Discussion Top


Intraocular schwannoma is commonly localized to the choroid (60%), followed by ciliary body and iris.[2] Schwannoma has no prototypical clinical or imaging features and has overlapping features with other intraocular tumors. Definitive diagnosis is achieved with HPE and immunohistochemistry. Alternating Antoni A and B areas and verocay bodies are characteristic histologic features.[3] Ancient schwannoma is a distinct subtype with degenerative features like cystic changes, hyalinized stroma, calcification, nuclear atypia, and low mitotic activity.[4] Diffuse immunoreactivity for S-100, lack of immunoreactivity to Melan-A and HMB 45, and lack of expression of smooth muscle actin and desmin help in differentiating schwannoma from neurofibroma, melanoma, and leiomyoma, respectively.

Intraocular schwannomas are benign and slow growing and can show local extension, oppression of surrounding structures, and extrascleral extension.[5] It can cause gradual visual loss, secondary glaucoma, and lead to a painful blind eye.[6]

In our case, clinical examination and ancillary imaging were inadequate in clinching a diagnosis. A well-timed incisional biopsy guided us toward the right diagnosis. A prompt and meticulous transcleral local resection of the slowly growing tumor helped preserving the globe with a visual acuity of 20//20. It is important to note that there are only 2 reported cases of ancient intraocular schwannoma.[7],[8] Our case is the only case where the globe was salvaged by local resection of the tumor.

The reported treatment options for intraocular schwannoma are observation, local excision, photodynamic therapy, intravitreal anti-vascular endothelial growth factor (VEGF), brachytherapy, and enucleation[1],[2],[5],[6],[7],[8],[9],[10] [Table 1].
Table 1: Reported cases of intraocular schwannoma and their management

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There is no optimal therapy yet. On reviewing the available literature, we recommend the following approach:

We can observe the tumor in cases that are stationary, with good visual acuity, and no secondary complications. Duration of follow-up should be tailored to individual cases.

In cases with expansile growth, good visual potential, and a pre-equatorial location, a local resection of the mass can be sight saving. With a high success rate and low complication and recurrence rate, local resection seems to be a rewarding option.

Intravitreal anti-VEGF and photodynamic therapy are potential options for tumors in unresectable locations and help preserve the globe. However, visual recovery is guarded. Further studies are warranted to study their effect. Brachytherapy seems unfavorable considering the low proliferative activity and abundance of collagen. Enucleation, except for painful blind eyes, should rarely be necessary in benign tumors like schwannoma.


  Conclusion Top


Uveal Schwannoma is a rare intraocular neoplasm with a predominantly benign course. Diagnosis can be challenging. An aspiration/incisional biopsy may help in diagnosis and avoid unnecessary enucleation in selected cases.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Shields JA, Sanborn GE, Kurz GH, Augsburger JJ. Benign peripheral nerve tumor of the choroid. A clinicopathologic correlation and review of the literature. Ophthalmology 1981;88:1322-9.  Back to cited text no. 1
    
2.
You JY, Finger PT, Iacob C, McCormick SA, Milman T. Intraocular schwannoma. Surv Ophthalmol 2013;58:77-85.  Back to cited text no. 2
    
3.
Kurtkaya-Yapicier O, Scheithauer B, Woodruff JM. The pathobiologic spectrum of schwannomas. Histol Histopathol 2003;18:925-34.  Back to cited text no. 3
    
4.
Canella C, Costa F, Klumb E, Aymoré IL, Marchiori E. Ancient schwannoma. Arthritis Rheum 2013;65(:2036.  Back to cited text no. 4
    
5.
Udyaver S, Lim L-AS, Milman T, Mashayekhi A, Shields JA, Shields CL. Intraocular schwannoma with extrascleral extension. Eur J Ophthalmol 2020;1120672120920211. doi: 10.1177/1120672120920211. Online ahead of print.  Back to cited text no. 5
    
6.
Jajapuram SD, Mishra DK, Kaliki S. Choroidal schwannoma presenting with neovascular glaucoma: A report of two cases. Oman J Ophthalmol 2019;12:125-8.  Back to cited text no. 6
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7.
Fan JT, Campbell RJ, Robertson DM. A survey of intraocular schwannoma with a case report. Can J Ophthalmol 1995;30:37-41.  Back to cited text no. 7
    
8.
Mortuza S, Esmaeli B, Bell D. Primary intraocular ancient schwannoma: A case report and review of the literature. Head Neck 2014;36:E36-8.  Back to cited text no. 8
    
9.
Damato B, Damato EM, Konstantinidis L, Heimann H, Coupland SE. Choroidal schwannoma: A case series of five patients. Br J Ophthalmol 2014;98:1096-100.  Back to cited text no. 9
    
10.
Yu Y, Cheng Y, Wang K, Sun K, Shen D, Liang J. Intraocular schwannoma: A case series of 3 patients. Oncol Lett 2019;17:1274-8.  Back to cited text no. 10
    


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