Nonsurgical treatment of an incompletely excised primary adenocarcinoma of nonpigmented ciliary epithelium

Primary adenocarcinoma of the nonpigmented ciliary epithelium (NPCE) is a very rare disease and the majority of acquired cases were treated by enucleation. We report the case of a 19-year-old man who had an adenocarcinoma arising from the NPCE. The tumor was found incidentally due to changed pupil shape. An excisional biopsy was performed and histopathological examination showed primary adenocarcinoma of the NPCE with a positive resection margin. Because of the positive resection margin and to save the eye, radiotherapy rather than enucleation was performed, followed by chemotherapy. Two years after the diagnosis, the patient continues to be followed up without evidence of relapse or cataract change. Our case suggests that adjunctive radiotherapy should be considered as a sight-saving treatment modality for primary malignant tumors of the NPCE.