Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019625 (Rosai-Dorfman disease)
 763 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A case of a 9-year-old Black African male with right-sided proptosis is presented. The clinical interpretation was that of orbital rhabdomyosarcoma and an incisional biopsy was performed. This revealed the typical features of Rosai-Dorfman disease, featuring S100 positive non-Langerhans histiocytes with emperipolesis. There were also numerous plasma cells in the biopsy, in the more fibrotic areas. More than 40% of these plasma cells were positive for IgG4. This case represents the first case in the orbit to make the association of IgG4 plasma cell positivity with Rosai-Dorfman disease. Potential associations are discussed, and the relevance of this finding to ophthalmic pathology interpretation and differential diagnosis are highighted.
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PMID:A case of orbital Rosai-Dorfman disease with IgG4 positive plasma cells. 2387 15

Extranodal Rosai-Dorfman disease involving the orbit has been reported severally with extensive discourse on management strategies. However, rhabdomyoblastic differentiation coexisting with the disease is yet unreported. We present the clinical and histopathological features of a 12-year-old boy presenting with both. A 12-year-old boy was referred to our Ophthalmic Plastic Surgical Service with a 5-year history of bilateral, progressively enlarging eyelid, orbital masses causing proptosis, and severe cosmetic blemish. He had suffered vision loss to the right eye following trauma 2 years prior to the onset of the disease. Computed tomography of the orbits revealed preseptal and intraorbital components of well-defined multi-lobulated masses in both orbits. No significant nodal involvement was noted. He underwent excision biopsy of the tumor in both eyes performed 1 month apart. A histopathologic diagnosis of the Rosai-Dorfman disease with focal areas of rhabdomyoblastic differentiation was made. He was treated postoperatively with a course of oral steroids with close observation for the rhabdomyoblastic differentiation. Rosai-Dorfman disease is an uncommon disease and has been reported to coexist with other pathologies. We highlight focal myoblastic differentiation within the excised tissues which may not necessarily warrant a diagnosis of rhabdomyosarcoma. Long-term follow-up of this patient is required to establish the safety of observation.
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PMID:Rhabdomyoblastic differentiation in rosai dorfman disease of the orbit in a 12-year-old male. 3056 Aug 34