A Rare Nonmalignant Mass of the Pancreas: Case Report and Review of Pancreatic Sarcoidosis MARTIN WIJKSTROM, M.D.,* RABIH I. BECHARA, M.D.,† JUAN M. SARMIENTO, M.D.* From the Departments of *Surgery and †Pulmonary Medicine and Critical Care, Emory University School of Medicine, Atlanta, Georgia Sarcoidosis is a systemic granulomatous disease of unknown etiology affecting patients from all genetic backgrounds. Pancreatic involvement is rare; the first case was described on autopsy in 1937. We present a case of pancreatic sarcoidosis without a history of the disease presenting as biliary obstruction mimicking pancreatic malignancy. We also review the literature with respect to management and outcomes of similar cases. The patient described here presented with all the signs and symptoms of a pancreatic malignancy, which was confirmed on a CT scan; the positron emission tomography scan and the CA 19-9 level were also confirmatory of the suspected di- agnosis. In this setting, if the mass looks resectable, a Whipple procedure would be the next logical step. However, such strategy would be aggressive management for a benign condition that could be palliated with diverting rather than resective procedures without changing the outlook of the disease. We suggest keeping a high index of suspicion in patients with a history of the disease if demographic concordance exists. S ARCOIDOSIS IS A systemic granulomatous disease of unknown etiology affecting patients from all ge- netic backgrounds. Although Th1-mediated immune responses involving interleukin-1, tumor necrosis fac- tor-a, and interferon-g have been implicated, neither the precipitating environmental trigger nor susceptible genotypes of patients have been identified. Recently, deficiencies in immune-regulatory T-cells have been implicated in the pathogenesis of sarcoidosis. Loss of immunoregulation by CD1d-restricted NKT cells, pro- tecting against disorders with increased CD4-positive Th1 responses in animals, may explain the amplified and persistent T-cell activity. 1 The T-cell subset CD4 + CD28 – is more abundant in patients with sar- coidosis, perhaps suggesting defective T-cell regula- tion. 2 Certain CTLA-4 polymorphisms are associated with multiorgan involvement. 3 Pancreatic involvement is rare; the first case was described on autopsy in 1937. 4 We present a case of pancreatic sarcoidosis without a history of the disease and presenting as biliary ob- struction mimicking pancreatic malignancy. Case Report A 49-year-old black woman without a significant medical or surgical history was found to have a pancreatic mass found on a CT scan dated November 2003 as part of a workup for a motor vehicle accident. On presentation in mid-December 2003, she reported a 1-month history of vague abdominal pain, pruritus, anorexia, and weight loss. She denied any fevers, chills, or night sweats. There was no history of tobacco or alcohol use. Family history was significant for one cousin with a 20-year history of sarcoidosis. Physical examination was normal; jaundice was not observed. Laboratory evaluation was consistent with biliary ob- struction: Total bilirubin 1.9 mg/dL (normal, 0.1–1.1 mg/dL), alkaline phosphatase 1045 U/L (normal, 30–110 U/L), alanine aminotransferase (ALT) 224 U/L (normal, 17–63 U/L), amylase 140 U/L (normal, 25–125 U/L), lipase 34 U/L (normal, 8–57 U/L), and CA 19-9 130 U/mL (normal, less than 37; using the Diagnostic Products Corporation Immulite 2000 GI- MA [CA 19-9] method). Autoimmune antibodies were negative, including antinuclear antibody, cytoplasmic, and antimyosin antibody. An initial dual-phase intravenous/by mouth contrast pancreatic-protocol CT scan revealed a hypodense pancreatic head mass measuring 4.4 3 4.1 cm without discrete margins in the superior aspect of the pancreas extending into the porta hepatis surrounding the he- patic artery and encasing two thirds of the portal vein circumference. It also demonstrated significant intra- hepatic and pancreatic ductal dilatation and relative at- rophy of the pancreatic body and tail parenchyma (Fig. 1). A CT-guided pancreatic biopsy was nondiagnostic. Address correspondence and reprint requests to Juan M. Sarmiento, M.D., Emory University Hospital, 1364 Clifton Road NE, Suite H-124C, Atlanta, GA 30322. E-mail: jsarmie@emory.edu. 79