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Extranodal Rosai-Dorfman Disease : Ileum

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Rosai-Dorfman disease (RDD) of the gastrointestinal tract: When involving the intestines, the presenting symptoms are non-specific and may include abdominal pain, constipation, and hematochezia. On colonoscopy, the lesion appears as a round, broad-based submucosal mass with intact overlying mucosa.

Grossly, the lesion is soft to firm, with a white-tan appearance. Areas of hemorrhage may be present. It is usually an infiltrative process extending into the muscularis propria, pericolic fat, and even adjacent lymph nodes.

This image of RDD of ileum (same case as previous slide) shows a diffuse infiltrate of histiocytes, lymphocytes, and plasma cells expanding the bowel wall.

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