Molecular Biomarkers & Diagnosis

Sarcoidosis Presenting with Primary Pancreatic Manifestations: A Case Report and Review of the Literature

Abstract

Eisar Al-Sukhni, Jingxin Qiu, Emmanuel Gabriel and Steven N Hochwald

Background: Pancreatic sarcoidosis in the absence of systemic sarcoidosis is a rare entity with few reported cases. Most described cases are in black females and typically involve the head of the pancreas. This report describes a case of sarcoidosis involving the tail of the pancreas in a Caucasian male.

Case Presentation: A previously healthy 48 year old Caucasian male presented with abdominal pain following an episode of heavy alcohol ingestion. Serum amylase was elevated and imaging was consistent with acute pancreatitis. Workup ruled out gallstones or autoimmune pancreatitis and his presentation was attributed to alcohol use. Despite abstaining from further alcohol intake, his abdominal pain persisted for several weeks, and subsequent imaging revealed persistent pancreatitis with narrowing of the pancreatic duct in the tail with an associated area of hypoechogenicity. FNA of this region showed rare benign ductal epithelial cells without evidence of malignancy. He was offered resection for his ongoing symptoms and to rule out a malignancy as an underlying etiology. He underwent laparoscopic distal pancreatectomy and splenectomy. Pathology from the resected specimen showed non-necrotizing granulomas involving the pancreas, spleen and the majority of 25 resected lymph nodes. Acid-fast bacteria and Gomori methenamine silver stains were negative for fungal or mycobacterial organisms and there was no evidence of polarizable materials within these granulomas. These findings are consistent with sarcoidosis. The patient recovered and was discharged without complication. He was subsequently referred to a rheumatologist for further workup and management.

Conclusions: Sarcoidosis can present with isolated pancreatic symptoms and may be difficult to distinguish from other causes of pancreatitis. In the absence of systemic disease, surgery alone may be both diagnostic and therapeutic.