Extrascleral Extension of Choroidal Melanoma Case 6

This tumor exhibits 3 signs consistent with ciliary body melanoma: displacement of the iris root, a sentinel vessel and extrascleral extension.

History

This 38 year-old patient was referred to The New York Eye Cancer Center with a large >16 mm base anterior uveal melanoma with a small plaque of extrascleral extension. 

Impression

Uveal Melanoma with Extrascleral Extension.

Note

This risks and benefits of observation, enucleation and radiation were discussed in detail. Primary enucleation was recommended and performed.

High frequency ultrasound (20 MHz) reveals displacement of the iris root (arrow). Though no sclerostomy is seen, the inner and outer scleral borders are poorly defined. 10 MHz ultrasonography shows an irregularly shaped tumor > 16 mm in diameter.

Comment

This case presents several classic findings in anterior uveal melanomas. Please share this case with your physicians in training. It is also important to note that not all ciliary body melanomas will exhibit these findings. Other findings of ciliary body melanomas include sector cataract and irregular astigmatism.

Lastly, there is some controversy about secondary radiation therapy as treatment for presumed residual microscopic orbital melanoma (due to extrascleral extension).

Without any compelling data to suggest it's efficacy, and knowing the significant morbidity associated with 50 Gy (typical dose) of external beam radiation therapy to the anophthalmic orbit (dry socket, lash-brow loss, and mucus discharge). This case is presented to The Eye Cancer Network's International Tumor Board for the opinions of ocular oncologists around the world. The voting is as follows.

Employ 50 Gy External Beam Radiation Therapy?

YES = 3
NO = 10

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