Rosai-dorfman disease (sinus histiocytosis with massive lymphadenopathy) of the pancreas: third reported occurrence.

Sinus histiocytosis with massive lymphadenopathy (SHML) was first described in 1969 by Rosai and Dorfman [1]. It is a rare inflammatory disorder with key clinicopathological characteristics such as emperipolesis and positive immunostaining for S-100 protein [2] and CD68. Rosai–Dorfman disease (RDD) is primarily manifested in the lymph nodes, yet extranodal disease has been reported in as many as half of patients [3]. Such cases affect primarily the head and neck, upper respiratory tract, and central nervous system, although the disease has been found in almost every organ system. The gastrointestinal tract is rarely involved, and only two cases of primary involvement of the pancreas have been previously described [4, 5]. Case Presentation