S2445 Unicentric Castleman Disease: A Case Report on a Rare Disease Entity

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Introduction: Unicentric Castleman’s Disease (UCD) is characterized by involvement of a single lymph node region and typically demonstrates an indolent course. Given the rarity of UCD and the nonspecific nature of the presenting features, the clinical presentation of lymphadenopathy and organ involvement may result in extensive evaluation for malignant or inflammatory/infectious aetiologies which delays diagnosis. Histologic examination is the key to a correct diagnosis. Case Description/Methods: Our case presents a 46-year-old man with no medical history who presented to the emergency room for persistent sharp epigastric pain. He denied alcohol consumption or tobacco use. He denied a family history of malignancy. On physical exam he was vitally stable with scleral icterus and epigastric tenderness without lymphadenopathy.Biochemical evaluation revealed a mixed pattern of elevated transaminases (aspartate aminotransferase-383, alanine transaminase-723, ALK-756) with direct hyperbilirubemia (3.9 mg/dl) and normal synthetic hepatic function. Lipase and alcohol level within normal limits. Initial computed tomography abdomen revealed abrupt cut off of common bile duct by a 2.7 x 2.7 pancreatic head mass with intrahepatic ductal dilatation for which Gastroenterology and General Surgery were consulted. Endoscopic retrograde cholangiopancreatography was performed with stent placement and brush biopsy. He was scheduled for a Whipple’s procedure which was aborted intraoperatively due to evidence of a mass encasing the portal vein. Histopathology confirmed the presence of Castleman’s Disease on the lymph node biopsy, and non-malignant inflammatory tissue with elevated IgG4 cells noted on pancreatic mass. Discussion: UCD is a rare condition of unknown aetiology with an indolent course. Definitive diagnosis is based on histopathology. The current standard of care for UCD is complete surgical resection once amenable, with high cure rates. However, radiation therapy is recommended for masses which cannot be completed resected. Chemotherapeutics, rituximab or anti–IL-6, are reserved for patients with UCD who cannot be treated with surgery or radiotherapy or for those who fail to respond to such treatment. Patients with UCD have excellent prognosis especially with successful treatment. In our case, we are faced with an unresectable for of UCD due to encasement of the portal vein which would qualify for a trial of radiation therapy. The uniqueness of our case is the presence of an increased ratio of IgG4 cells in the histopathology of the pancreatic mass which suggests a relationship between these 2 disease entities and provokes further investigation.

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