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With the exception of melanocytomas, ciliary body nevi are rare. Previous case series have reported episcleral nodular extension of ciliary body nevi leading to their diagnosis1 or alternatively ciliary body nevi with involvement of the iris root, resulting in their detection using slitlamp biomicroscopy.2 Herein, we report a pigmented limbal nodule consistent with a ciliary body nevus diagnosed post mortem in a donor eye as an incidental finding during corneoscleral disc preparation for transplantation.
Report of a Case
We received an enucleated eye from a male organ donor in his early 50s. It had a separated corneoscleral disc and a single well-circumscribed brown-pigmented limbal nodule. The donor kidneys had already been transplanted, and hence there was the concern of potential malignancy of this lesion while preparing the cornea for storage in the eye bank. The requested histomorphological examination of the remaining enucleated eye showed autolytic changes only, without any evidence of a choroidal melanoma. The corneoscleral disc contained anterior ciliary body muscle, trabecular meshwork, and cornea. A small flat tumor embedded in the ciliary body was observed, composed of homogeneous spindle cells that were pigmented with ovoid nuclei and without any cellular atypia (Figure 1). These cells extended along transscleral blood vessels into the episclera, where they formed a 2 × 0.5-mm nodule. Immunohistochemistry demonstrated melan A– and S-100 protein–positive spindle cells, with a Ki-67 proliferation rate less than 1%. Molecular genetic analysis by multiplex ligation-dependent probe amplification of both the intraocular and extraocular components of the lesion showed normal patterns for chromosomes 1p, 3, 6, and 8 (Figure 2); no GNAQ or GNA11 mutations were detected by sequencing. Taken together, these results were consistent with the histomorphological diagnosis of a ciliary body nevus with involvement of the episcleral conjunctiva.
A, Corneoscleral disc showing ciliary body nevus with transscleral extension into the episclera (original magnification ×3.4, hematoxylin-eosin). The area outlined by the box is shown in B. B, Intrascleral extension of bland nevus cells (original magnification ×40, hematoxylin-eosin). C, Melan A–positive nevus cells in the episcleral nodule (original magnification ×3.4, 3,3'-diaminobenzidine immunostaining). D, S-100 protein–positive nevus cells (original magnification ×40, 3,3'-diaminobenzidine immunostaining).
Results of multiplex ligation-dependent probe amplification of the ciliary body nevus. The loci examined are indicated by their gene names on the x-axis. The parallel dashed lines indicate the range within which the loci are considered to be normal for uveal melanoma. The overall status for chromosomes 1p, 3, 6, and 8 was considered to be normal for this case.
This case is rare. To our knowledge, there has been no previous description of a ciliary body nevus in a donor eye with transscleral extension.
The differential diagnoses of a single well-circumscribed episcleral brown-pigmented nodule include conjunctival melanocytic neoplasias, conjunctival pigmented squamous tumors, traumatic pigment inclusions, extraocular extension of a uveal nevus (the most common pseudomelanoma3), or a uveal melanoma. The dominance of the small tumor in this case within the ciliary body and its associated bland histomorphological features (also seen in the extraocular component) spoke for the earlier-mentioned diagnosis and against the other differential diagnoses. This was supported by the results of the molecular genetic analysis of the tumor for chromosomes 1p, 3, 6, and 8, which proved to be consistent with a benign melanocytic tumor. The unusual transscleral extension observed is reminiscent of that seen in hyperpigmented magnocellular nevi (also termed melanocytomas)4 and also occasionally in cases of bilateral diffuse uveal melanocytic proliferation.
Because the kidneys of this organ donor had already been transplanted into 2 patients prior to the preparation of both donor eyes for corneal transplantation, there was considerable concern that the donor had an undiagnosed ocular melanoma that had potentially already metastasized and could be a danger for the organ recipients, as previously described.5 As a result of our histomorphological and molecular genetic findings, we were confident that the transplanted kidneys did not require explantation, although special follow-up of the recipients was recommended.
Eye donor selection guidelines in Europe and the United States allow systemic malignant neoplasms (except hematological) to be present provided there is no ocular involvement, but ocular malignant neoplasms are contraindications to corneal transplantation.6 Our case suggests that thorough posterior segment examination of all donor eyes also be considered.
In conclusion, this case presents an unusual finding in an eye from an organ donor and highlights the need for careful examination of all donor eyes prior to corneal transplantation.
Corresponding Author: Sarah E. Coupland, MBBS, PhD, FRCPath, Department of Molecular and Clinical Cancer Medicine, Institute of Translational Medicine, University of Liverpool, Sixth Floor, Duncan Bldg, Daulby Street, Liverpool L69 3GA, England (firstname.lastname@example.org).
Published Online: March 19, 2015. doi:10.1001/jamaophthalmol.2015.214.
Conflict of Interest Disclosures: All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest and none were reported.
Nissen E, Armitage J, Thornton S, Coupland SE. Pigmented Limbal Nodule Consistent With a Ciliary Body Nevus in an Organ Donor. JAMA Ophthalmol. 2015;133(6):721–723. doi:10.1001/jamaophthalmol.2015.214
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