Isolated Intraocular Rosai-Dorfman Disease

• Published in: Ocular oncology and pathology 2019-10, Vol.5 (6), p.418-423
• Main Authors: Fogt, Franz, Rüediger, Thomas, Augustin, Albert J., Frank, Dale M., Rosenwald, Andreas, Wellmann, Axel, Lee, Vivian
• Format: Article
• Language: English
• Subjects: Novel Insights from Clinical Practice
• ISSN: 2296-4681; 2296-4657
• DOI: 10.1159/000497185

Background/Aims: To report a case of Rosai-Dorfman disease (RDD) presenting as a solitary, choroidal mass, initially suspicious for uveal melanoma, in a 72-year-old woman. Methods: Retrospective case report of a single patient. Results: A 72-year-old woman presented with sudden vision loss in the right eye. A month prior, visual acuity was 20/40, but she was noted to have a choroidal mass confirmed with B-scan ultrasonography. Patient’s vision deteriorated significantly a month later and a shallow retinal detachment was newly noted. Magnetic resonance imaging was obtained, demonstrating a hyperintense intraocular tumor on TI imaging. Patient underwent enucleation of the right eye for suspicion of a uveal melanoma. Pathology revealed a mixed cellular infiltrate with histiocytes, some exhibiting emperipolesis. Macrophage immunohistochemical stains were positive, while melanocytic markers were negative. A diagnosis of RDD was made. Subsequently, the patient had a negative workup for systemic involvement. A final diagnosis of intraocular RDD without extraocular and systemic involvement was determined. Conclusion: We describe a rare presentation of RDD as a solitary choroidal mass in an elderly patient with overlapping features of uveal melanoma. Definitive diagnosis could only be made on histology. RDD should be considered in the differential diagnosis of a choroidal lesion in the elderly.