Rosai-Dorfman disease masquerading as Uveal Melanoma: Case report and review of literature

Publication date: Available online 15 June 2018

Source: Hematology/Oncology and Stem Cell Therapy

Author(s): Yacoub A. Yousef, Maysa Al-Hussaini, Rashed Nazzal, Ghadeer Abdeen, Ibrahim Alnawaiseh, Khaleel Alrawashdeh

Abstract

Objective/Background

Intra ocular Rosai-Dorfman disease (RDD) is an extremely rare disease. We are reporting the first case of RDD presenting as ciliary body mass mimicking ciliary body melanoma, and we are reviewing the English literature reporting on cases of RDD presented with intraocular disease.

Methods

An 18-year-old lady presented with loss of vision in the right eye, and was found to have intraocular mass lesion. She was diagnosed clinically and radiologically as a case of ciliary body melanoma associated with total retinal detachment.

Results

Histopathological sections and stains proved to be intraocular RDD. Review of the literature revealed three cases of intraocular RDD; two of them had choroid thickening associated with serous retinal detachment, and one presented with intraocular mass mimicking choroid melanoma. Two of the three cases were enucleated. Our case is the first case in English literature of intraocular ciliary body RDD, mimicking ciliary body melanoma.

Conclusion

RDD can present as an intraocular mass that mimics ciliary body melanoma. This case emphasizes the importance of diagnostic biopsy before considering the final therapy in unclear cases, mainly when associated with unusual systemic features like lymphadenopathy.

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