Large Pancreatic Cyst in Von Hippel-Lindau (VHL) Syndrome Causing Bile Duct Obstruction

Introduction: Von Hippel-Lindau (VHL) syndrome is a rare autosomal inherited disorder characterized by the development of multiple tumors, the most common of which is renal cell carcinoma, hemangioblastomas, pheochromocytomas, and solid and cystic tumors of the pancreas. Morbidity and mortality in VHL are more commonly related to renal and central nervous system pathologies. Extrahepatic biliary obstruction secondary to pancreatic cysts is rare in patients with VHL. We present a case of extrahepatic biliary obstruction secondary to pancreatic cysts in a patient with a history of VHL syndrome successfully managed endoscopically. Case Description/Methods: 35-year-old woman with history of VHL and course complicated by clear cell renal cell carcinoma status post partial nephrectomy, cervical/medullary/fourth ventricle hemangioblastomas status post resection, and serous cystic pancreatic adenoma status post distal pancreatectomy and splenectomy with subsequent development of insulin-dependent diabetes mellitus type 2, presented with three days of right upper quadrant abdominal pain. MR Cholangiopancreatography revealed the residual pancreas was completely replaced by multiple cystic lesions with a dominant cyst in the head of the pancreas measuring 6.2 cm causing mass effect on the portal vein and mid to distal common bile duct with resultant intrahepatic, extrahepatic biliary ductal dilatation, and gallbladder distension. Using EUS with fine-needle biopsy of the cyst was done. Intraprocedural cholangiogram during ERCP revealed 14mm dilation of proximal extrahepatic biliary duct and non-opacification of the distal extrahepatic bile duct in the region of the pancreatic head, consistent with a stricture. CBD brushings were sent for cytology and a straight plastic biliary stent was successfully deployed across the stricture. After the endoscopic procedure, the patient demonstrated clinical improvement. Discussion: The pancreatic manifestation of VHL can present as a cystic, solid, or combined lesion. The management is complex because of the challenge in the differentiation of benign cystic lesions from malignant lesions. This case highlights the fact that pancreatic involvement leading to acute biliary obstruction, although uncommon, can occur in patients with VHL syndrome. Conservative management with endoscopic biliary stent placement and endoscopic surveillance (for secondary manifestations - portal hypertension) may be helpful in these patients, however eventual surgical management may be needed.Figure 1.: (Top left) Stricture/obstruction in ERCP, (Bottom left) EUS with biopsy, (Right) MRI showing cyst, CBD, and distended gallbladder.