Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) of the pancreas: First case report

Rosai-Dorfman Disease is a histiocytic proliferative disorder which primari ly affects lymph nodes. Extranodal involvement occurs in about one half of the patients and the head and neck area represents the region most commonly involved. We present the case of a 48 year-cold female who was found with. a pancreatic mass during evaluation for abdominal pain. She underwent a di stal pancreatectomy and splenectomy. Her pathology showed sinus histiocytos is with massive lymphadenopathy (SHML) involving the pancreas and lymph nod es and focally the spleen. The histiocytes characteristically contained one or more viable lymphocytes in the cytoplasm. The lymphocytes had penetrate d the cytoplasm in a process known as '"emperipolesis", where the lymphocyt es continued to have free movement in the histiocyte. The histiocytic cells were positive with S-100 protein and CD68. Rosai-Dorfman Disease (SHML) ca n affect the peripancreatic lymph nodes with possible secondary pancreatic involvement and present as a pancreatic mass.