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Int J Clin Exp Pathol 2013;6(11):2569-2577

Case Report
Extranodal Rosai-Dorfman disease involving appendix and mesenteric nodes with a
protracted course: report of a rare case lacking relationship to IgG4-related disease and
review of the literature

Ming Zhao, Changshui Li, Jiangjiang Zheng, Jingjing Yu, Hongcun Sha, Minghui Yan, Jie Jin, Ke Sun, Zhaoming Wang

Department of Pathology, Ningbo Yinzhou Second Hospital, Ningbo, Zhejiang Province, PR China; Department of General Surgery, Ningbo
Yinzhou Second Hospital, Ningbo, Zhejiang Province, PR China; Departmen of Radiology, Ningbo Yinzhou Second Hospital, Ningbo, Zhejiang
Province, PR China; Department of Pathology, The First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, Zhejiang
Province, PR China

Received September 1, 2013; Accepted September 23, 2013; Epub October 15, 2013; Published November 1, 2013

Abstract: Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare disease of unknown
etiology that typically presents as nodal disease in young children. However, it also can present in various extranodal sites and can be difficult
to recognize if not considered in the differential diagnosis. Here, we report a case of appendix involvement by extranodal RDD, which occurred
in a 69-year-old woman with a long duration of 12 years for intermittent right lower quadrant pain. The patient underwent a right hemicolectomy
for a clinical diagnosis of appendiceal cancer. A mixed inflammatory infiltration of mature lymphocytes, plasma cells and histiocytes exhibiting
emperipolesis were indentified. Other areas had storiform fibrosis and sclerosis admixed with numerous plasma cells. These histologic
features combination with immunoreactivity for CD68 and S100 protein were indicative of a diagnosis of extranodal RDD. We discuss the
clinical, pathologic findings as well as differential diagnoses and consideration of a possible relationship of this entity to IgG4-related lesion.
(IJCEP1309001).

Keywords: Rosai-Dorfman disease, extranodal disease, gastrointestinal tract, appendix, IgG4-related disease, differential diagnosis

Address correspondence to: Dr. Ming Zhao or Dr. Changshui Li, Department of Pathology, Ningbo Yinzhou Second Hospital, No.1 North
Qianhe Road, Yinzhou District, Ningbo, Zhejiang 315100, PR China. Tel: +86-574-83039018; Fax: +86-574-83038515; E-mail:
zhaomingpathol@163.com (Ming Zhao); lcs333@163.com (Changshui Li)