Rosai–Dorfman Disease (Sinus Histiocytosis with Massive Lymphadenopathy) of the Pancreas: Second Case Report

Abstract

She underwent a Whipple procedure. Her pathology of both the pancreatic mass and RML lung wedge resection showed sinus histiocytosis with massive lymphadenopathy, along with extensive fibrosis intertwined with nodular mixed inflammatory infiltrate. The histiocytes characteristically showed "emperipolesis," in which lymphocytes had penetrated the cytoplasm and remained viable within the histiocytes (lymphocytes continued to have free movement in the histiocyte). In addition, the histiocytic cells were positive with S-100 protein and CD68, hallmarks of RDD. Although rare, Rosai-Dorfman disease should be considered in the differential diagnosis of patients presenting with pancreatic and/or lung nodules, especially when biopsy or cytology results report atypical inflammatory findings.

Rosai–Dorfman Disease (Sinus Histiocytosis with Massive Lymphadenopathy) of the Pancreas: Third Reported Occurrence

“…Esquivel first reported a 48-year-old African–American female with a 3.5-cm mass in the body of the pancreas, who underwent a distal pancreatectomy. This patient did not have lymphadenopathy, and preoperative biopsies had been inconclusive [5]. Zivin reported a second case in 2009, a 63-year-old African–American female with obstructive jaundice and weight loss, who was found to have a mass in the head of the pancreas.…”

“…Zivin reported a second case in 2009, a 63-year-old African–American female with obstructive jaundice and weight loss, who was found to have a mass in the head of the pancreas. Preoperative fine-needle aspiration was negative for malignancy [5]. …”

“…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]. …”

Rosai‐Dorfman disease of the pancreas: Cytologic analysis of three cases presenting as pancreatic masses

“…Although originally described in lymph nodes, RDD may also involve extranodal sites. Pancreatic involvement is extremely rare, with few cases reported in the literature 8‐17 . In this study we describe the clinicopathologic and cytologic features as well as diagnostic challenges encountered in three patients with Rosai‐Dorfman disease of the pancreas.…”

Rosai–Dorfman disease with diffuse gastrointestinal involvement

“…Most reported extranodal sites for RDD are skin, upper respiratory tract, bone, genitourinary system, central nervous system, orbit, and soft tissues [2]. The gastrointestinal (GI) system is one of the least commonly affected sites, with several reported cases of liver, pancreas, and solitary or segmental involvement of the GI tract [3][4][5][6][7][8][9][10][11]. Establishing a correct and timely diagnosis is difficult in patients with extranodal involvement because of variable clinical manifestations in different organ or system involvements.…”