title: Rosai-Dorfman disease presenting as choroidal melanoma: a case report and review of the literature.
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.
authors.affiliation: Anatomical Pathology, PathWest, Royal Perth Hospital, Wellington Street, Perth 6000 Western Australia, Australia. tersia.vermeulen@health.wa.gov.au
authors.forename: Tersia L
authors.initials: TL
authors.lastname: Vermeulen
authors.forename: Timothy W
authors.initials: TW
authors.lastname: Isaacs
authors.forename: Dominic
authors.initials: D
authors.lastname: Spagnolo
authors.forename: Benhur
authors.initials: B
authors.lastname: Amanuel
mesh_terms: D000328:Adult; D014408:Biomarkers, Tumor / Q000032:analysis; D002830:Choroid Neoplasms / Q000737:chemistry / Q000175:diagnosis*; D003937:Diagnosis, Differential; D015353:Eye Enucleation; D005451:Fluorescein Angiography; D015618:Histiocytosis, Sinus / Q000175:diagnosis*; D006801:Humans; D008297:Male; D008545:Melanoma / Q000737:chemistry / Q000175:diagnosis*; D012163:Retinal Detachment / Q000175:diagnosis; D014792:Visual Acuity / Q000502:physiology
chemical_list: D014408:Biomarkers, Tumor