S2650 Rosai-Dorfman Disease of the Pancreas Mimicking Pancreatic Cancer

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Introduction: Rosai-Dorfman disease (RDD) is a rare benign non-Langerhans histiocytic disorder described as an accumulation of histiocytes which usually presents as bilateral painless cervical lymphadenopathy with associated symptoms of fever, weight loss, and night sweats and can have extranodal manifestations. GI manifestations are generally rare but carry a 20% mortality rate. Although RDD is usually self-limited, treatment focuses typically on immunosuppressive therapies. This case report highlights a unique presentation of RDD with an extranodal GI manifestation. Case Description/Methods: An 85-year-old man with a past medical history of atrial fibrillation on warfarin therapy presented to the emergency department with melena and complaint of epigastric pain. Computed tomography (CT) imaging and endoscopy did not show any acute findings. Endoscopic ultrasound was done which showed a 4 cm pancreatic mass (Figure 1A) that stained positive for S100 on histologic analysis consistent with RDD (Figure 1B). He was discharged from the hospital on a steroid taper and later returned with worsening symptoms with repeat CT imaging showing intrahepatic and extrahepatic biliary duct dilation, 2cm dilated common bile duct and isodense pancreatic mass. The patient underwent endoscopic retrograde cholangiopancreatography with metal stent placement with resolution of his symptoms (Figure 1C). Discussion: RDD presents a difficult diagnostic picture given the varying spectrum of presentations. Classically, this ailment is seen with symptoms of weight loss, night sweats, and fever. This case is unique as the patient had no classic symptoms and instead presented with concerns of autoimmune hepatitis. Histologically, RDD histiocytes exhibit emperipolesis and S100 staining. Literature on the gastrointestinal (GI) manifestations of RDD is limited, with the GI tract being involved in less than 1% of all extranodal cases and having a higher mortality rate as compared to non-GI cases. Treatment for RDD usually depends on the sequelae of the nodal or extra-nodal manifestation present. Although there are no current guidelines on treatment of RDD, a suggested approach is 6-12 months of systemic therapy followed by observation. Generally, response is assessed and a steroid taper can be started with escalation to immunomodulator therapy with TNF-alpha inhibitors such as lenalidomide if the patient is not improving. Although usually self-limited, the difficulty of diagnosis of RDD can lead to delayed treatment.Figure 1.: A) Endoscopic ultrasound with a 4 cm hypoechoic well-defined mass in the neck of the pancreas. B) Direct smears from the endoscopic ultrasound-guided fine needle aspiration/fine needle biopsy of the mass showed numerous histiocytes with intermixed groups of pancreatic acinar cells (A – Papanicolaou stained slide, 200X; B – Diff-Quik stained slide, 400X). C) Endoscopic retrograde cholangiopancreatography with common bile duct stricture.

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