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Figure 1.
Clinical photographs. A, Clinical photograph shows the anterior chamber angle with gonioscopy. A pigmented mass is present, extending into the anterior chamber in the superotemporal quadrant. B, Clinical photograph through a dilated pupil shows a pigmented mass in the ciliary body region indenting the lens superotemporally.

Clinical photographs. A, Clinical photograph shows the anterior chamber angle with gonioscopy. A pigmented mass is present, extending into the anterior chamber in the superotemporal quadrant. B, Clinical photograph through a dilated pupil shows a pigmented mass in the ciliary body region indenting the lens superotemporally.

Figure 2.
Ultrasound biomicroscopy (UBM). A, UBM shows a ciliary body mass (T) with cystic spaces. The inner sclera adjacent to the base of the tumor shows a hypoechoic area (arrows). B, The hypoechoic area on UBM is revealed by low-power photography on hematoxylin-eosin (H-E)–stained section as an area with pale eosinophilic staining (arrows). Scale bar, 0.5 mm. C, At high power (H-E), the sclera immediately adjacent to the tumor shows feathery and irregular collagen fiber appearance (arrows) in contrast to unaffected sclera where collagen fibers appear in regular bundles. Scale bar, 250 μm.

Ultrasound biomicroscopy (UBM). A, UBM shows a ciliary body mass (T) with cystic spaces. The inner sclera adjacent to the base of the tumor shows a hypoechoic area (arrows). B, The hypoechoic area on UBM is revealed by low-power photography on hematoxylin-eosin (H-E)–stained section as an area with pale eosinophilic staining (arrows). Scale bar, 0.5 mm. C, At high power (H-E), the sclera immediately adjacent to the tumor shows feathery and irregular collagen fiber appearance (arrows) in contrast to unaffected sclera where collagen fibers appear in regular bundles. Scale bar, 250 μm.

1.
van den Hooff  A The part played by the stroma in carcinogenesis. Perspect Biol Med 1984;27 (4) 498- 509
PubMed
2.
Alyahya  GAHeegaard  SPrause  JU Characterization of melanoma associated spongiform scleropathy. Acta Ophthalmol Scand 2002;80 (3) 322- 326
PubMedArticle
3.
Alyahya  GARibel-Madsen  SMHeegaard  SPrause  JUTrier  K Melanoma-associated spongiform scleropathy: biochemical changes and possible relation to tumour extension. Acta Ophthalmol Scand 2003;81 (6) 625- 629
PubMedArticle
4.
Alyahya  GA Melanoma associated spongiform scleropathy: characterization, biochemical and immunohistochemical studies. Acta Ophthalmol. 2008;86(thesis 3):1-21
PubMed
5.
Weisbrod  DJPavlin  CJEmara  KMandell  MA McWhae  JSimpson  ER Small ciliary body tumors: ultrasound biomicroscopic assessment and follow-up of 42 patients. Am J Ophthalmol 2006;141 (4) 622- 628
PubMedArticle
6.
Noble  JQazi  FALakosha  HK  et al.  Extrascleral extension in association with ciliochoroidal melanoma: ultrasound biomicroscopy with histopathological correlation. Can J Ophthalmol 2005;40 (5) 616- 618
PubMedArticle
Citations 0
Research Letters
August 2009

Clinical Detection of Melanoma-Associated Spongiform Scleropathy by Ultrasound Biomicroscopy and Its Correlation With Pathological Diagnosis

Arch Ophthalmol. 2009;127(8):1064-1066. doi:10.1001/archophthalmol.2009.162

Ciliary body and choroidal melanoma account for greater than 90% of all uveal melanomas. Extrascleral extension is an important prognostic factor in uveal melanoma and has been described in 8% of eyes enucleated in the Collaborative Ocular Melanoma Study. Infiltrating solid tumors may cause cellular and degenerative changes in connective tissue surrounding the tumor, contributing to tumor cell invasion.1 For these reasons, characterization of scleral changes adjacent to the tumor may be relevant to tumor invasion.

Melanoma-associated spongiform scleropathy (MASS) is a histopathological entity described as an area within the sclera adjacent to a choroidal or ciliary body melanoma where collagen fibers appear to have disintegrated into loose fibers.2 Melanoma-associated spongiform scleropathy is observed in approximately one-third of eyes with uveal melanoma.2,3 Biochemical analyses of MASS show decreased collagen and amino acids and increased glycosaminoglycans and water uptake. This degradation process, mediated by matrix metalloproteinases,3 may weaken the structural barrier and facilitate local tumor invasion. Alyahya4 observed MASS in 91% of eyes with tumor invasion, in contrast to 23% of eyes without. We report for the first time the clinical detection of MASS by ultrasound biomicroscopy (UBM) in ciliary body melanoma, with histopathological correlation.

Report of a Case

A 44-year old man presented with painless decreased vision in the right eye for several months. Visual acuity was 20/25, and on dilated examination a melanocytic ciliochoroidal mass was identified abutting the lens (Figure 1A). Gonioscopy showed a pigmented mass invading the anterior chamber angle (Figure 1B).

B-scan ultrasound showed a large dome-shaped mass involving the ciliary body that measured 5.4 mm in thickness. There were cystic spaces in the lesion. A-scan demonstrated medium reflectivity with a decrescendo pattern. Ultrasound biomicroscopy showed intralesional cavities. The inner one-fourth of the sclera adjacent to the base of the tumor was hypoechoic compared with the outer three-fourths of the sclera (Figure 2A). The thickness of the sclera over the lesion was deemed to be normal.

Diagnosis of a medium-sized, malignant, ciliochoroidal melanoma was made, and enucleation was performed.

Gross examination showed a pigmented superotemporal ciliary body mass measuring 6 × 9 × 4 mm. The ciliary body melanoma was composed of spindle B and epithelioid melanoma cells, with large cavities containing eosinophilic exudate. Tumor cells were noted in the anterior chamber angle and iris root (Figure 2B) and extended to the inner sclera only. The inner sclera adjacent to the tumor showed a pale staining area relative to the outer sclera and neighboring scleral regions (hematoxylin-eosin staining [H-E], Figure 2B). High-power examination showed a feathery appearance of the inner sclera and dot morphology of collagen fibers in contrast to the normal interlacing collagen fiber bundles in the surrounding sclera (H-E, Figure 2C). The diagnosis was ciliary body melanoma with MASS.

Comment

Ultrasound biomicroscopy has been a valuable tool in the detection and management of anterior segment and ciliary body tumors.5 Careful assessment of UBM characteristics of the tumor-scleral interface enables the detection of intrascleral invasion and small extrascleral tumor extension.6 Owing to the irregular arrangement of its collagen fibrils, the sclera produces ultrasound backscatter that results in uniform high reflectivity. The region of lower reflectivity involving the inner scleral layers adjacent to the tumor corresponded to MASS seen histopathologically. The observed low reflectivity likely relates to loose arrangement of collagen fibrils and increased water content. As MASS is a noninflammatory degradation process of scleral collagen that may facilitate tumor invasion, this observation may lead to larger studies to assess whether the clinical detection of MASS by UBM might have clinical or prognostic significance.

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Article Information

Correspondence: Dr Yücel, Ophthalmic Pathology Laboratory, University of Toronto, St Michael's Hospital, 30 Bond St, Shuter Wing 5-038, Toronto, ON M5B 1W8, Canada (yeni.yucel@utoronto.ca).

Financial Disclosure: None reported.

References
1.
van den Hooff  A The part played by the stroma in carcinogenesis. Perspect Biol Med 1984;27 (4) 498- 509
PubMed
2.
Alyahya  GAHeegaard  SPrause  JU Characterization of melanoma associated spongiform scleropathy. Acta Ophthalmol Scand 2002;80 (3) 322- 326
PubMedArticle
3.
Alyahya  GARibel-Madsen  SMHeegaard  SPrause  JUTrier  K Melanoma-associated spongiform scleropathy: biochemical changes and possible relation to tumour extension. Acta Ophthalmol Scand 2003;81 (6) 625- 629
PubMedArticle
4.
Alyahya  GA Melanoma associated spongiform scleropathy: characterization, biochemical and immunohistochemical studies. Acta Ophthalmol. 2008;86(thesis 3):1-21
PubMed
5.
Weisbrod  DJPavlin  CJEmara  KMandell  MA McWhae  JSimpson  ER Small ciliary body tumors: ultrasound biomicroscopic assessment and follow-up of 42 patients. Am J Ophthalmol 2006;141 (4) 622- 628
PubMedArticle
6.
Noble  JQazi  FALakosha  HK  et al.  Extrascleral extension in association with ciliochoroidal melanoma: ultrasound biomicroscopy with histopathological correlation. Can J Ophthalmol 2005;40 (5) 616- 618
PubMedArticle
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