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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 1  |  Issue : 4  |  Page : 745-748

Melanoma-associated spongiform scleropathy: A case report


1 Department of Ocular Pathology, Uveitis and Neuro-Ophthalmology, Sri Sankaradeva Nethralaya, Guwahati, Assam, India
2 Department of Oculoplasty, Cataract and Refractive Surgery, Sri Sankaradeva Nethralaya, Guwahati, Assam, India
3 Department of Vitreo-Retina, Sri Sankaradeva Nethralaya, Guwahati, Assam, India
4 Department of Physician, Sri Sankaradeva Nethralaya, Guwahati, Assam, India
5 Department of Ophthalmology, Sri Sankaradeva Nethralaya, Guwahati, Assam, India
6 Ocular Pathology, Sri Sankaradeva Nethralaya, Guwahati, Assam, India

Date of Submission11-Feb-2021
Date of Acceptance13-Jun-2021
Date of Web Publication09-Oct-2021

Correspondence Address:
Dr. Dipankar Das
Department of Ocular Pathology, Uveitis and Neuro-Ophthalmology, 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/ijo.IJO_337_21

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  Abstract 


Melanoma-associated spongiform scleropathy (MASS) is a non-inflammatory scleral degenerative change with a spongiotic morphology seen in the sclera associated with uveal melanoma. This degenerative change was seen in the inner side of the sclera in contact with the pigmented mass. It is usually seen in an elderly person with choroidal melanoma. We report a case of MASS in a large choroidal melanoma in a 58-year-old Indian man supported by histopathology, special histochemical stains, and immunohistochemistry.

Keywords: Choroid, enucleation, melanoma, scleropathy


How to cite this article:
Das D, Bhattacharjee K, Barman MJ, Bharali G, Javeri H, Zameer S, Buragohain S, Mahato DK, Gokani B, Deka A. Melanoma-associated spongiform scleropathy: A case report. Indian J Ophthalmol Case Rep 2021;1:745-8

How to cite this URL:
Das D, Bhattacharjee K, Barman MJ, Bharali G, Javeri H, Zameer S, Buragohain S, Mahato DK, Gokani B, Deka A. Melanoma-associated spongiform scleropathy: A case report. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Oct 21];1:745-8. Available from: https://www.ijoreports.in/text.asp?2021/1/4/745/327736



Choroidal melanoma accounts for 80% of uveal melanoma.[1],[2],[3],[4],[5],[6],[7] Melanoma-associated spongiform scleropathy (MASS) is a cellular and degenerative change in the adjoining sclera seen in intraocular melanoma, which causes a biochemical change in the scleral collagen.[1],[2],[5],[6],[7] MASS is observed in about one-third of eyes with uveal melanoma, more so in large and extra-large ones.[1],[2],[5],[6],[7] It has been observed that around 92% may show scleral extension in choroidal melanoma cases. MASS may facilitate tumor extension.[2],[3],[4],[5],[7] We report a case of MASS with large melanoma in an elderly patient with supportive evidence of histopathology and histochemical staining.


  Case Report Top


A 58-year-old Indian man reported to a tertiary institute of northeast India with diminution of vision in the right eye (OD) for 18 months. He was diagnosed elsewhere as neovascular glaucoma with retinal detachment in OD. Perception of light was negative in OD and left eye (OS) had vision 20/30 partial, N8 and corrected to 20/20, N6 with refraction. On slit-lamp examination, the anterior segment was quiet in both eyes with neovascularization of iris in OD. Intraocular pressure by applanation tonometry was 43 mmHg in OD and 10 mmHg in OS, respectively. The angle of the anterior chamber was closed in OD in all quadrants and open in OS. There was a dense cataract in OD because of which the fundus was not visualized. Fundus examination was normal in OS. B-scan ultrasound in OD revealed a choroidal mass (14.20 × 7.10 × 6.80 mm) in the superior-temporal quadrant with moderate internal reflectivity suggestive of choroidal melanoma [Figure 1]. Magnetic resonance imaging showed a hyperintense, mild homogenously enhancing choroidal melanoma in the superior lateral aspect of the right globe projecting into the vitreous cavity. Investigations which included chest X-ray PA view, whole abdomen ultrasound, liver function test, complete blood count, random blood sugar, serum creatinine, and electrocardiogram were within normal limits. Screening for retrovirus and serology for hepatitis were negative. After proper consent, enucleation of the OD with silicon ball implantation was carried out under local anesthesia.
Figure 1: B-scan ultrasound in the right eye revealing a choroidal mass (14.20 × 7.10 × 6.80 mm) in the superior-temporal quadrant with moderate internal reflectivity suggestive of choroidal melanoma. Please note the incremental decrease of vector A-scan spikes (positive Kappa angle) within the growth

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Gross examination showed normal measurements and transillumination defect was seen [Figure 2]a and the eyeball was sectioned vertically in the plane based on the transillumination test. The cornea was normal. Minimal exudative retinal detachment was seen. A brownish colored tumor was seen with a collar-stud appearance [Figure 2]b.
Figure 2: (a) Showing transillumination defect and eyeball was sectioned vertically in the plane based on the transillumination test. (b) Showing cut section of the eyeball with a brownish colored tumor measuring 14.35 × 7.45 mm having collar-stud appearance. Exudative retinal detachment was noted in the specimen

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Microscopic examination showed a normal cornea with a deep anterior chamber. The bleached section of the tumor showed both epithelioid and spindle B-cells [Figure 3]a. AJCC, 8th edition grading system was employed to grade this tumor- pT3, pN0, pM0 with histological grading of G2 (>10% epithelioid cells and <90% spindle cells): Mixed cells, spindle A and B with few epithelioid cells.
Figure 3: (a) Bleached section of the tumor with both epithelioid and spindle B-cells type (H and E, ×40×). (b) Spongiform scleral change consistent with MASS (arrows) (H and E, ×40×)

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Scleral fiber degeneration was also noted in the posterior sclera and pigmented cells were seen in the scleropathy areas. Spongiform scleral change was also seen [Figure 3]b and these scleral fiber degeneration particularly spongiform change were seen with Alcian blue stain [Figure 4]a. Scleral degeneration was also confirmed by Masson's trichrome stain [Figure 4]b and periodic acid Schiff' (PAS) stain. No extra-scleral spread was observed. Clumps of pigmented cells were seen adjoining the area of exit of the vortex vein. Histopathological diagnosis of choroidal melanoma (OD), mixed cell type without extra scleral spread was made. Scleral fiber degeneration showed MASS change. Immunohistochemistry (IHC) showed HMB 45 positive (++) [Cell Marque, USA] for melanoma cell and Ki67 labeling index showed growth fraction between 20% and 50%[Cell Marque, USA].
Figure 4: (a) Showing glycosaminoglycans deposition on the sclera with Alcian blue stain (arrow) (×40×). (b) Showing scleral degeneration in Masson's trichrome stain (arrow) (×20×)

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The patient was doing well postoperatively and a prosthesis was fitted at 6 weeks. Metastatic work-up was negative for the patient.


  Discussion Top


Choroidal melanoma can be associated with MASS.[1],[2],[5],[6],[7] MASS is a non-inflammatory condition seen in choroidal melanoma with secondary change in the sclera where disintegrated collagen fibers are loosely attached.[2],[3],[5] It is seen in around 38% of cases of melanomas of the choroid and ciliary body.[2],[3],[5] It has been postulated that scleral change is proportional to the contact between the pigmented tumor and adjoining sclera.[2],[3],[4],[5],[6] MASS can facilitate the tumor to have a scleral extension and it requires further validity in pathological samples.[2],[3],[5] In large studies, MASS has been found to be reduced in pre-enucleation radiation, especially in the older patients.[2],[3],[5] Our patient did not receive any form of radiation before enucleation. Cell type of choroidal melanoma can be spindle-A cells, spindle-B cells, epithelioid cells, and mixed types as seen in our case.[1] However, the mass could not be correlated with choroidal melanoma cell types.[2],[3],[5] MASS showed lower levels of amino acids and protein content indicating collagen degradation.[2],[3],[5]

In many cases of MASS, the extra-scleral spread can be present.[1],[2],[5],[7] Biochemically, there are defects of amino acids' side chain, collagen I, and proteins in the sclera, resulting in spongiform change.[1],[2],[5],[7] In our case, the patient presented with a right-sided painful blind eye due to neovascular glaucoma, and large choroidal melanoma was diagnosed on radiological investigations. Enucleated eyeball showed mixed-type melanoma and spongiotic scleral change was seen without extra-scleral spread. However, malignant melanoma cells were seen on the exit of the vortex veins. Special histochemical stains were very useful in the study of the scleral change.[1],[2],[5],[7] Glycosaminoglycans (GAG), which were picked up by Alcian blue stain, were found to be abnormally deposited on the scleral fibers and they were expressed in MASS, particularly in low pH (2.0) Alcian blue stain. GAG was seen to be expressed in an increased amount in scleral tissue in MASS in the previous studies.[1],[2],[5],[7] Masson's trichrome along with PAS stain showed scleral stromal degeneration.[1],[2],[5],[7] Type I collagen and total protein reduction in the sclera were seen in the previous studies.[1],[2],[5],[7] We, however, did not perform the chemical analysis of scleral tissue in our case. An interesting study on matrix metalloproteinase (MMPs) showed overexpression of MMP-2 in the scleral component with MASS;[7] in which it was postulated that deranged scleral fibers caused the overexpression of MMP-2.[7] Our case had moderate expression (20%–50%) of a proliferative marker in IHC for the KI67 labeling index, which was correlated with the presence of epithelioid cells in the tumor having vortex vein involvement.


  Conclusion Top


This unique feature of pigmented cell migration in the sclera in scleropathy area is important as scleropathy might suggest a weak point in the sclera due to loose scleral fiber attachment which might help in the pigment cell migration or formation of cellular tract in the sclera. This was not previously documented in the scientific literature. More observations of MASS with similar changes are required in future pathological studies.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent form. In the form, the patient had given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initial will not be published and due efforts will be made to conceal his identity.

Acknowledgements

Sri Kanchi Sankara Health and Educational Foundation.

Dr. Hemalata Deka MS; Dr Himadri S. Das, MD; Dr Pradip Hatimota, MD; Mr. Pankaj Kumar Goswami, MSc.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Robert F. The sclera. In: Heegaard S, Grosssniklaus HE, editors. Eye Pathology: An Illustrated Guide. Berlin Heidelberg: Spinger-Verlag; 2015. p. 155-72.  Back to cited text no. 1
    
2.
Alyahya GA. Melanoma-associated spongiform scleropathy. Characterization, biochemical and immunohistochemical studies. Acta Ophthalmol 2008;86:1-21.  Back to cited text no. 2
    
3.
Rao R, Honavar SG, Mulay K. Multifocal choroidal melanoma in oculodermal melanocytosis in an Asian Indian. Indian J Ophthalmol Case Rep2019;67:2089-91.  Back to cited text no. 3
    
4.
Alyahya GA, Heegaard S, Prause JU. Characterization of melanoma associated with spongiform scleropathy. Acta Ophthalmol Scand 2002;80:322-6.  Back to cited text no. 4
    
5.
Alyahya GA, Ribel-Madesen SM, Heegaard S, Prause JU, Trier K. Melanoma-associated spongiform scleropathy: Biochemical changes and possible relation to tumour extension. Acta Ophthalmol Scand 2003;81:625-9.  Back to cited text no. 5
    
6.
Weisbrod D, Pavlin CJ, McGowan H, Yucel YH. Clinical detection of melanoma-associated spongiform scleropathy by ultrasound biomicroscopy and its correlation with pathological diagnosis. Arch Ophthalmol 2009;127:1064-6.  Back to cited text no. 6
    
7.
Alyahya GA, Kolko M, Prause JU, Nielsen BS, Wang J, Heegaard S. Matrix matalloproteinase-2 is expressed in melanoma associated spongiform scleropathy. Invest Ophthalmol Vis Sci. 2008;49:2806-11.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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