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Rosai-Dorfman Disease of the Digestive System—Beware Vasculopathy

A Clinicopathologic Analysis

Alruwaii, Zainab I. MD; Zhang, Yang MD; Larman, Tatianna MD; Miller, James A. MD, MPH; Montgomery, Elizabeth A. MD

The American Journal of Surgical Pathology: December 2019 - Volume 43 - Issue 12 - p 1644–1652
doi: 10.1097/PAS.0000000000001343
Original Articles

Rosai-Dorfman disease (RDD) is a rare non-Langerhans cell histiocytic proliferation that occurs in nodal and extranodal sites. Rare examples of the disease involving the digestive system have been described. To characterize the digestive tract manifestations of this disease, 12 specimens from 11 patients with extranodal RDD affecting the digestive organs were analyzed. Hematoxylin and eosin sections and available immunohistochemical stains were reviewed, and the clinical information was obtained from patients’ electronic or submitted records. Eight patients were female and 3 male (median age, 65 y; range, 17 to 76 y). Abdominal pain was the most frequent symptom. Six patients had an associated immunologic or malignant disease. Nine lesions arose in the gastrointestinal tract (1 involving the appendix, 2 right colon, 6 left colon), 2 in the pancreas, and 1 in the liver. Two patients had the coexistent nodal disease, and 1 had bone and soft-tissue involvement. The lesions were generally composed of polygonal to spindle-shaped histiocytes with eosinophilic to clear cytoplasm admixed with lymphoplasmacytic cells. The inflammatory cells formed lymphoid aggregates in 7 cases and included focally scattered or small collections of neutrophils in 6 cases. Fibrosis was variable, and 4 cases had a storiform pattern. Vasculopathy in the form of a thickened capillary wall, medium-sized arterial wall infiltration by lesional and inflammatory cells and phlebitis was seen in 10, 5, and 2 cases, respectively. All cases were reactive for S100-protein. Of the 5 patients with follow-up, 1 developed immunoglobulin A nephropathy and died of renal failure.

Department of Pathology, Johns Hopkins Medical Institutions, Baltimore, MD

Conflicts of Interest and Source of Funding: The authors have disclosed that they have no significant relationships with, or financial interest in, any commercial companies pertaining to this article.

Correspondence: Elizabeth A. Montgomery, MD, Department of Pathology, Johns Hopkins Medical Institutions, Weinberg 2242, 401 North Broadway, Baltimore, MD 21231 (e-mail:

Online date: August 12, 2019

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