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Rosai–Dorfman disease presenting as choroidal melanoma: a case report and review of the literature

  • Pathology
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Abstract

Background

Rosai–Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare non-malignant proliferation of histiocytes of unknown aetiology. It was first recognised as a distinct clinicopathologic entity in 1969, and is classified as an idiopathic non-Langerhans cell histiocytosis. The disease process is usually self-limiting and often involves lymph nodes, but extranodal involvement is well-described and any anatomic site can be involved.

Methods

We describe a unique case of a 40-year-old male who presented with a fundus mass diagnosed clinically as choroidal melanoma. The tumour showed rapid growth. The patient developed a total retinal detachment and underwent enucleation. The globe contained a choroidal tumour with histologic and immunophenotypic features characteristic of RDD. The literature of ocular Rosai–Dorfman disease was reviewed.

Results

This is the first case in the English literature of intraocular choroidal RDD, mimicking choroidal melanoma.

Conclusions

Rosai–Dorfman disease can present as a mass-producing lesion in the choroid and may mimic other choroidal tumours. The case emphasises the need to consider diagnostic biopsy prior to definitive treatment of choroidal tumours.

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Authors and Affiliations

  1. Anatomical Pathology, PathWest, Royal Perth Hospital, Wellington Street, Perth, 6000, Western Australia, Australia

    Tersia L. Vermeulen

  2. Lions Eye Institute, Nedlands, Perth, Australia

    Timothy W. Isaacs

  3. Anatomical Pathology, PathWest, Queen Elizabeth II Medical Centre, Nedlands, Western Australia, Australia

    Dominic Spagnolo & Benhur Amanuel

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  1. Tersia L. Vermeulen
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  2. Timothy W. Isaacs
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  3. Dominic Spagnolo
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Correspondence to Tersia L. Vermeulen.

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Vermeulen, T.L., Isaacs, T.W., Spagnolo, D. et al. Rosai–Dorfman disease presenting as choroidal melanoma: a case report and review of the literature. Graefes Arch Clin Exp Ophthalmol 251, 295–299 (2013). https://doi.org/10.1007/s00417-012-2175-5

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  • DOI: https://doi.org/10.1007/s00417-012-2175-5

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