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Jackelien van Beek, Anna Koopmans, Jolanda Vaarwater, Annelies de Klein, Robert Verdijk, Emine Kilic, Rotterdam Ocular Melanoma Study; Extraocular extension in uveal melanoma. Invest. Ophthalmol. Vis. Sci. 2013;54(15):4245.
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In approximately 13% of all enucleated uveal melanoma patients, extraocular spread is found. It is reported that the size of the intraocular tumour is related to extraocular extension and to metastatic death. Extraocular spread, irrespective of which route, is correlated with histopathologic and cytogenetic factors of increased malignancy. Hence in this study we have identified all cases of uveal melanoma with extraocular extension and investigated prognostic factors and correlation with survival.
In this retrospective study, initiated by the Rotterdam Ocular Melanoma Study (ROMS) group, the Netherlands, patients were included if they were referred with a choroidal or ciliary body uveal melanoma from 1987 until 2011. Only patients were included who underwent primary or secondary enucleation due to a choroidal of ciliary body melanoma.
In total 356 patients were included, with a mean age of 60.6 years at moment of diagnosis (range 21-91 years). Extraocular extension was confirmed by revision of the histopathological coupes in 43 patients (67.4% male, mean age: 63.7 years). Extraocular spread occurred in 34 patients with choroidal melanoma, in 8 with ciliary body melanoma and in 1 patient with a ciliary body and choroidal melanoma localisation. The mean basal diameter was 14.2 mm (range: 6-22 mm) with a mean prominence of 7.7 mm (range: 1 - 22 mm). Five times an epithelioid cell type, 22 times a mixed cell type and 16 times a spindle cell type was observed. Extraocular extension occurred via perivascular invasion (41 patients), direct scleral invasion (33 patients), perineural invasion (29 patients) and invasion through Schlemm’s canal (6 patients). Most of the extraocular extensions followed a combination of these routes, 4 patients had only vascular spread and 1 patient had only neural spread. The (largest) periscleral diameter of the extraocular extension ranged from 0.1 mm to 40 mm, with a mean of 2.86 mm. In 19 out of 37 tumors with extraocular extension, monosomy 3 was observed. Of the 43 patients with extraocular extension, 20 are alive, of which 4 with metastases. There were 19 patient deaths, of which 16 had metastases.
Presence of extraocular extension was not related with histological cell type. Even though 44% of the tumors with extraocular extension had monosomy 3, metastasis was observed in only 47%. Nevertheless, extraocular extension was associated with a worse survival (p=0.019).
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