Abstract

Introduction: Rosai-Dorfman disease (RDD) is a benign, proliferative disease of non-Langerhans cell histiocytes typically involving lymphoid tissue. Pancreatic involvement is extremely rare.
Case Description: Our case is of a 53-year-old female who originally presented with vague epigastric abdominal pain. Magnetic resonance imaging (MRI) revealed a mass in the pancreatic body suspicious for malignancy. The patient successfully underwent a robotic-assisted subtotal pancreatectomy and splenectomy. The final pathology revealed sheets of foamy histiocytes with enlarged vesicular nuclei and nucleoli. Immunohistochemical testing was positive for S-100 protein and CD163 and negative for CD1a and ALK-1, establishing a diagnosis of pancreatic Rosai-Dorfman disease. Next generation sequencing was positive for an F53L variant mutation of the MAP2K1 gene.
Discussion: Overall, there is no uniform approach for RDD treatment, treatment is usually tailored to the individual’s clinical picture. Here we describe a novel technique of robotic-assisted resection of pancreatic Rosai-Dorfman disease.

Key words: Rosai-Dorfman Disease; pancreas; robotic subtotal pancreatectomy; histiocytes