Acquired neoplasms of the nonpigmented ciliary epithelium (adenoma and adenocarcinoma)

Abstract

Background/purpose: Acquired neoplasms of the nonpigmented ciliary body epithelium (NPCE) are rare, and most information about them has come from single case reports. This study was undertaken to review the authors' experience with a series of patients with acquired neoplasms of the NPCE, to delineate the clinical and histopathologic features of these tumors, and show how they differ from ciliary body melanoma.

Methods: A clinicopathologic review was conducted on acquired tumors of the NPCE that were evaluated by the authors and a review of the English language literature was done. The data from the authors' cases were compared with previously reported cases.

Results: The authors had personal experience with nine patients with acquired tumors of the NPCE and found 18 other patients with these tumors in the literature. Of the authors' patients, all tumors were predominantly nonpigmented and were white to light-tan in color. Associated clinical findings included signs of intraocular inflammation in all patients, secondary cataract in eight (89%), and subluxation of the lens in six (67%). Eight of the tumors were managed successfully by local resection and one by enucleation. Histopathologically, the tumors showed considerable variation from patient to patient. Seven tumors were classified as benign adenoma and two as low-grade adenocarcinoma. There was no local recurrence or systemic metastases. Although tumors of the NPCE historically have been misdiagnosed clinically as ciliary body melanoma, our study suggests that they have some characteristic features that severe to differentiate them from melanoma and other ciliary body lesions. In contrast to melanoma, acquired neoplasms of the NPCE are amelanotic and are more likely to have an irregular surface, associated inflammatory signs, to transmit light well during transillumination, and show high internal reflectivity with ultrasonography.

Conclusion: Acquired neoplasms of the NPCE have characteristic clinical and histopathologic features that should suggest the diagnosis. Due to their anterior location in the ciliary body, local resection (rather than enucleation) is usually the treatment of choice. The visual prognosis is fair, and the systemic prognosis is excellent.