Melanoma-Associated Spongiform Scleropathy in Oculodermal Melanocytosis with Primary Orbital Melanoma

Abstract

Purpose: To describe spongiform scleropathy in a patient with oculodermal melanosis and without evidence of uveal melanoma.

Methods: Clinical-pathological correlation conducted in compliance with HIPPA (Health Insurance Privacy and Portability Act) regulations.

Results: Melanoma-associated spongiform scleropathy was an incidental finding in an 87-year-old woman with oculodermal melanocytosis treated for primary orbital melanoma. All previously reported cases of this scleropathy have been associated with uveal melanoma.

Conclusions: The mechanism of scleral degeneration in melanoma-associated spongiform scleropathy is unknown, and its clinical and prognostic significance is speculative. This is the first case of a so-called melanoma-associated spongiform scleropathy reported in an eye without uveal melanoma.

Keywords: Melanoma-associated spongiform scleropathy; Melanosis oculi; Nevus of Ota; Oculodermal melanosis; Scleral degeneration.

Fig. 1

Axial computed tomography showing a large intraconal tumor extending to the apex of the right orbit. Inset With the lid elevated, brown episcleral pigmentation is seen.

Fig. 2

Low magnified view of the eye and retrobulbar connective tissue showing melanoma in the lower left corner. This is as close as the orbital melanoma approached the globe. The retina was detached during processing. The choroid is modestly thick and heavily pigmented. Scleral abnormalities cannot be appreciated at this magnification (hematoxylin-eosin; bar = 1,600 µm). Inset Cross-sections of the optic nerve reveal no direct tumor involvement or heterotopic melanocytes (hematoxylin-eosin; bar = 900 µm).

Fig. 3

Three hematoxylin-eosin-stained sections showing melanoma-associated spongiform scleropathy involving the inner third of the sclera. The left panel shows a heavily pigmented, uniformly thick nevus of the choroid and abnormal inner scleral collagen (arrowheads; bar = 300 µm). The middle panel at higher magnification reveals the wispy, shredded wheat appearance of the collagen in longitudinal section (bar = 80 µm). The right panel shows splayed and fragment collagen that makes up the fascicles. The degenerative changes impart weak eosin uptake (bar = 35 µm).

Fig. 4

The melanin-bleached section from the choroid showing faint nuclei of thin, spindle-shaped melanocytes among bleached granules. The nuclei of melanocytes are almost imperceptible, consisting of a thin nuclear envelope and with almost no stainable chromatin (bar = 30 µm).