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Extranodal Rosai-Dorfman Disease : Ileum

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Gastrointestinal tract is one of the least commonly affected extranodal site in Rosai-Dorfman disease (RDD). Most cases are discovered incidentally or found at autopsy. The locations have included colon, ileum, appendix, pancreas, and liver.

A large proportion of cases have disseminated disease with nodal or extranodal involvement. RDD of the gastrointestinal tract in not a self-limiting disease (unlike cases involving a single lymph node region) and pursues a protracted clinical course. Treatment is with surgery and/or systemic corticosteroid therapy.

This image shows RDD involving terminal ileum in a young male. The small bowel lumen and ileal mucosa can be seen on the upper left. Lower half shows expansion of the bowel wall by an infiltrate of histiocytes, lymphocytes, and plasma cells.

Reference: Anders R. A., Keith J. N., & Hart J. Rosai-Dorfman Disease Presenting in the Gastrointestinal Tract. Arch Pathol Lab Med. 2003;127:e74-e75

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