B0073 - Pancreatic Rosai-Dorfman Disease Diagnosed Without Surgery

Mark Hsu, MD, Katrina Naik, MD, Amith Subhash, MD, Jose Aponte-Pieras, MD, Annie Hong, MD, Yousif Elmofti, MD, Muhammad Talha Farooqi, MD, Shahid Wahid, MD
Kirk Kerkorian School of Medicine at UNLV, Las Vegas, NV

Introduction: Rosai-Dorfman disease (RDD) is a benign non-Langerhans cell histiocytosis that rarely involves the pancreas, of which only eighteen cases have been published to our knowledge. At least fourteen of these cases required surgical intervention. Here we present a case of pancreatic RDD diagnosed by endoscopic ultrasound with fine needle aspiration (EUS-FNA) only, thus avoiding surgical intervention for the patient.

Case Description/Methods: A 49-year-old female with diabetes and hypertension presented with intermittent abdominal pain. CT scan of the abdomen showed a 4cm pancreatic body mass and a 2.3cm retroperitoneal mass. Pathology from EUS-FNA biopsies of both masses showed findings consistent with RDD. This included histiocytosis with emperipolesis, stains positive for S100, CD68, and CD163, and negative for CD1a. There were no signs of carcinoma or IgG4-related disease. Repeat CT imaging 2 years later demonstrated progressive growth of the masses. The pancreatic mass grew to 4.4cm and the retroperitoneal mass grew to 3.3cm. Repeat EUS-FNA biopsies from both sites once again demonstrated pathologic findings consistent with RDD. The patient agreed to continue routine surveillance without surgical intervention given her stable symptoms.

Discussion: Most cases of pancreatic RDD have required surgical intervention for diagnosis due to initially nonspecific EUS or CT-guided biopsies. Some of these initially nonspecific biopsies were shown to have findings of RDD on re-review of pathology. While there are currently no consensus guidelines for RDD treatment, a wide array of nonsurgical options exists such as observation (as in our case), immunomodulatory agents, and chemotherapy. Increased awareness of RDD can potentially avoid unnecessary surgical intervention, thus affecting patient mortality and morbidity. We propose that pancreatic RDD does not need surgical intervention for diagnosis, and in cases of nonspecific inflammation on pathology without obvious evidence of malignancy, re-review of the pathology with a focus on RDD should be considered before surgical intervention.




Disclosures:


Mark Hsu, MD, Katrina Naik, MD, Amith Subhash, MD, Jose Aponte-Pieras, MD, Annie Hong, MD, Yousif Elmofti, MD, Muhammad Talha Farooqi, MD, Shahid Wahid, MD. B0073 - Pancreatic Rosai-Dorfman Disease Diagnosed Without Surgery, ACG 2022 Annual Scientific Meeting Abstracts. Charlotte, NC: American College of Gastroenterology.