Characterization of melanoma associated spongiform scleropathy

Abstract

ABSTRACT.Purpose: Melanoma associated spongiform scleropathy (MASS) describes areas within the sclera where collagen bundles appear to have disintegrated into loose fibres. It is found adjacent to choroidal and ciliary body melanoma. This study aimed to characterize these changes histopathologically. Methods: Sections of 218 eyes that had been enucleated for malignant melanoma between January 1994 and June 2000 were examined for MASS, using data collected from the files of the Eye Pathology Institute, University of Copenhagen. Findings were correlated to patient characteristics (age, gender and pre-enucleation radiation) and tumour characteristics (location, morphology and invasion). Staining was applied using haematoxylin-eosin (HE), periodic acid-Schiff (PAS), haematoxylin-phloxine-saffron (HPS), alcian blue, colloidal iron and Masson's trichrome. Sixteen normal eyes and 29 eyes with diseases other than choroidal and ciliary body melanoma served as controls.Results: Melanoma associated spongiform scleropathy was observed in 73 of the 218 eyes (33%) with melanoma. No changes were found in normal eyes or in eyes with other intraocular diseases. A significantly higher incidence of MASS was found in older age groups. Thirty-six (49%) of the 73 cases of MASS were observed within a patient age range of 71-90 years, whereas only 14 cases (19%) were observed within an age range of 41-60 years. Pre-enucleation radiation significantly reduced the incidence of MASS. Twenty-two (10%) of the total 218 eyes had received pre-enucleation radiation and in only two (1%) of these was MASS observed. Melanoma associated spongiform scleropathy was found only in areas of contact between the tumour and the sclera, and the degree of MASS correlated directly with the extent of this contact. No significant correlation with tumour cell type was found. Conclusions: In eyes with MASS, the severity of the changes correlated to the extent of direct contact between the tumour and sclera. Melanoma associated spongiform scleropathy showed a significant predilection towards older age groups. Pre-enucleation radiation significantly reduced the incidence of MASS. Melanoma associated spongiform scleropathy is probably caused by a deposition of amorphous material splitting the scleral collagen fibrils. This material may be secreted either directly from the adjacent tumour or from scleral fibroblasts stimulated by the tumour cells.

Melanoma-Associated Spongiform Scleropathy Adjacent to a Choroidal Nevus

“…There is an apparent paradoxical observation that specific and total glycoasaminoglycans are found in higher concentrations within melanoma-associated scleropathy than normal sclera through biochemical assay [5], yet when histochemical stains for mucosubstances and polysaccharides are applied, there is reduced stainability [1]. Our findings are consistent with reports of reduced reactivity of Alcian blue, PAS, and colloidal iron in the area of scleral degeneration (Fig.…”

“…Our findings are consistent with reports of reduced reactivity of Alcian blue, PAS, and colloidal iron in the area of scleral degeneration (Fig. 4) [1]. Further insights into the physical-chemical properties of this conditions are needed to clarify this apparent contradiction of findings.…”

“…Although the pathogenesis of melanoma-associated spongiform scleropathy is not well understood, it is considered a condition found exclusively with posterior uveal melanoma, as its name explicitly indicates. One study of 218 eyes with choroidal and ciliary body melanoma found 73 tumors with melanoma-associated spongiform scleropathy (33%) [1]. That study also included 45 normal and tumor-bearing eyes which did not display the scleropathy.…”

“…A peculiar degenerative change of scleral collagen associated with contiguous melanoma of choroid and/or ciliary body was initially reported by Stefani at the 1989 European Ophthalmic Society meeting [1]. The condition was called melanoma-associated spongiform scleropathy and has been subsequently found in 11–33% of eyes harboring choroidal and ciliary body melanomas [1-3].…”

“…The condition was called melanoma-associated spongiform scleropathy and has been subsequently found in 11–33% of eyes harboring choroidal and ciliary body melanomas [1-3]. The condition is thought specific to choroidal-ciliary body melanoma and has not been described in benign tumors or metastatic cancers to the eye.…”