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Year : 2021  |  Volume : 64  |  Issue : 4  |  Page : 839-841
A rare case of intraocular epithelioid leiomyoma


1 Department of Ocular Pathology, Sri Sankaradeva Nethralaya, Guwahati, Assam, India
2 Department of Ophthalmology, Sri Sankaradeva Nethralaya, Guwahati, Assam, India

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Date of Submission28-May-2020
Date of Decision19-Jun-2020
Date of Acceptance27-Jul-2020
Date of Web Publication20-Oct-2021
 

How to cite this article:
Das D, Kuri GC, Magdalene D, Barman M, Talukdar HK, Das BC, Deshmukh S, Gupta P, Deka A. A rare case of intraocular epithelioid leiomyoma. Indian J Pathol Microbiol 2021;64:839-41

How to cite this URL:
Das D, Kuri GC, Magdalene D, Barman M, Talukdar HK, Das BC, Deshmukh S, Gupta P, Deka A. A rare case of intraocular epithelioid leiomyoma. Indian J Pathol Microbiol [serial online] 2021 [cited 2021 Dec 7];64:839-41. Available from: https://www.ijpmonline.org/text.asp?2021/64/4/839/328569




A 8-year-old boy presented to a tertiary institute of northeast India with the chief complaint of dimness of vision in the right eye (OD) for 7 months which was gradual and worsening. Past history revealed diagnosis of complicated cataract and total retinal detachment in OD. There was a history of injury with iron stick of umbrella 7 months back. On examination, vision in OD was perception of light negative and left eye (OS) was 20/20, N6. Slit-lamp examination showed conjunctival congestion and corneal vascularization in OD. Neovascularization of iris and cataractous lens was observed in OD. Dilated fundus examination revealed no view in OD and OS was normal. B-scan ultrasound of OD showed decreased axial length with normal lens echo but lens could not be focused properly. Suspected inferior peripheral choroidale was seen. There was gross increase in retino-choroidal thickness with phthisical change in OD. Vitreo-retina clinic was consulted next. As OD was blind, enucleation with silicon ball implant was advised after parental consent and it was carried out by Oculoplasty surgeon. Enucleated eyeball was subjected for pathological examination in the ocular pathology laboratory.

Grossly, enucleated eyeball (OD) measured normally in all dimensions. Optic nerve was cut flush to the surface. Transillumination defect was not seen. Eyeball was sectioned vertically. Retro-lental grayish-white colored mass measured 10 mm X 16 mm was noted which was oval and circumscribed [Figure 1].
Figure 1: Gross enucleated specimen showing retro-lental grayish-white mass (marked with arrow)

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Microscopic examination in hematoxylin-eosin stained slide showed corneal edema with stromal neovascularization and scarring. Anterior chamber was deep. Iris showed isolated neovascularization. Angle was closed in one end and opened at the other. There was lenticular degeneration with isolated calcification noted. Disorganization of ciliary body was observed. Retro-lental mass was noted with infiltration by vesicular looking cells with spindle cells [Figure 2]a and [Figure 2]b. Cells showed occasional interlacing, tightly packed bundle of spindle- shaped cells, and absence of collagenous stroma. Some of the nuclei were oval and vesicular. Cells arranged in palisading pattern. There was no mitosis seen in the section studied. Retinal exudation was observed with normal choroid and sclera. Impression of intraocular epithelioid leiomyoma of ciliary body was made. Special stain with Masson Trichrome showed differential staining [[Figure 3]a, 20x]. Immunohistochemistry (IHCs) by kit method revealed Vimentin +++ [[Figure 3]b, 20x], Desmin +++ [[Figure 3]c, 20x] and Smooth Muscle Actin (SMA) ++ [[Figure 3]d, 20x] and negative for S-100 [[Figure 3]e, 20x] and CK [[Figure 3]f, 20x]. Proliferative marker Ki 67 was 1+. IHCs were carried out by CELL MARQUE, USA. IHCs Slides were compared with the controls.
Figure 2: (a) X 100, H, and E; showing spindle-shaped cellular tumor arising from the ciliary body region. (b) X 400, H and E; showing prominent vesicular and spindle-shaped cells in the tumor

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Figure 3: X 200, Special stain (a-trichrome) showing differential staining and IHCs (b-Vimentin+++), (c-Desmin+++) and (d-Smooth muscle actin++). IHCs for S-100 (e) and CK (f) were negative for the tumour

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Intraocular leiomyoma involves the uveal tract. Leiomyoma in human body occurs mostly in females.[1],[2],[3] Uveal leiomyoma commonly occurs in 3rd and 4th decade of life..[1],[2],[3],[4] Often they are located in supraciliary and suprachoroidal space where they transmit light and often described to have sparkling transillumination defect.[2],[3],[4],[5],[6] Pathologically, tumor is benign and cells are arranged in tightly packed bundles having occasional interlocks.[3],[4],[5],[6] Many of the cells' nuclei can be oval or vesicular and the cells were mostly arranged in palisading pattern.[3],[4],[5] They can be stained with Masson Trichrome, Phosphotungstic acid-hematoxylin (PTAH) stain and by Periodic acid-Shiff (PAS) stain for basement membrane.[3],[4],[5] There are different varieties of leiomyomas and one such atypical variety is called mesectodermal leiomyoma which can be seen in ciliary body also.[3],[4] Histopathologically and by IHCs, they show both myogenic and neurogenic characteristics.[3],[4],[5]

Our case was atypical on few aspects. First, it was seen in young boy whereas most of the leiomyomas are seen after the 3rd decade and common in females. Second, the tumor had predominance of vesicular cells that led to the diagnosis of epitheloid leiomyoma which again has rare intraocular occurrence. Third, foci of lenticular calcification were noted in our case which has not been reported previously in the literature.

In genetic analysis study, most of the leimyomas of human body have normal karyotyping but can have some chromosomal abnormalities such as in chromosome 12q14 and 6p that codes for HMCK and HMG1Y gene respectively.[6] Recently, MED 12 mutation was identified in uterine leiomyomas and such studies for ocular leiomyomas can be done in the future.[6]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient's parent consent forms. In the form the patient, father has given his consent for boy's images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity.

Acknowledgements

Kanchi Sankara Health and Educational Foundation, Guwahati, Assam, India.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Shields JA, Shields CL, Eagle RC Jr, De Potter P. Observations on seven cases of intraocular leiomyoma. The 1993 Byron Demorest lecture. Arch Ophthalmol 1994;112:521-8.  Back to cited text no. 1
    
2.
Biswas J, Kumar SK, Gopal L, Bhende MP. Leiomyoma of the ciliary body extending to the anterior chamber: Clinicopathologic and ultrasound biomicroscopic correlation. Surv Ophthalmol 2000;44:336-42.  Back to cited text no. 2
    
3.
Eagle RC Jr. Intraocular tumors in adults. In: Eagle RC Jr, editor. Eye Pathology. An Atlas and Text. 2nd ed. Philadelphia, Pa: Lippincott Williams and Wilkins; 2011. p. 195-7.  Back to cited text no. 3
    
4.
Shields JA, Shields CL, Eagle RC. Mesectodermal leiomyoma of the ciliary body managed by partial lamellar iridocyclochoroidectomy. Ophthalmology 1989;96:1369-76.  Back to cited text no. 4
    
5.
Tomar AS, Finger PT, Iacob CE. Intraocular leiomyoma: Current concepts. Surv Ophthalmol Surv Ophthalmol 2020;65:421-37.  Back to cited text no. 5
    
6.
Mittal P, Shin YH, Yatsenko SA, Castro CA, Surti U, Rajkovic A. Med12 gain-of-function mutation causes leiomyomas and genomic instability. J Clin Invest 2015;125:3280-4.  Back to cited text no. 6
    

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Correspondence Address:
Dipankar Das
Department of Ocular Pathology, Uveitis and Neuro-Ophthalmology Services, Sri Sankaradeva Nethralaya 96 Basistha Road, Beltola, Guwahati, Assam
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_622_20

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