Noncommunicating Mucinous Biliary Cystadenoma: A Rare Cause of Jaundice

Abstract

Introduction: Biliary cystadenomas (BCA) represent a rare cystic biliary neoplasm with the potential for malignant transformation. A limited number of cases of BCA have been reported worldwide. Two types have been described: mucinous and serous. BCA with ovarian-like stroma is a characteristic of mucinous cystadenomas and mainly affects women. Their clinical presentation is non-specific, including abdominal pain and fullness, nausea, increased abdominal girth, and, rarely, a palpable mass. We report a rare case of noncommunicating mucinous BCA presenting as painless jaundice. A 58-year-old healthy female presented with jaundice, pruritus, elevated liver enzymes, and dilated bile duct on abdominal US. MRI showed intrahepatic duct dilation and a cystic dilation of the common hepatic duct. ERCP revealed a stricture at the common hepatic duct near the bifurcation, cholangioscopy, and directed biopsies were unremarkable. Placement of a biliary stent resulted in a significant clinical improvement and resolution of lab abnormalities. The patient underwent endoscopic ultrasound, which showed an exophytic cyst compressing the common hepatic duct. Ten cc of mucinous fluid was aspirated with FNA. MRI showed a decreased size of the cystic lesion at the porta hepatis. ERCP was repeated to exchange the biliary stent. Cholangiogram showed luminal narrowing at the left hepatic duct take off with a fixed, round opacification occluding 75% of the lumen, and proximal to this was a second stricture where the lumen narrowed up to 95%. Due to the persistence of biliary obstruction, a laparoscopic common bile duct excision with cholecystectomy and choledochoduodenostomy was performed. A mass adherent to the anterior wall of common bile duct was removed. Histology showed a polycystic mucinous cystadenoma with ovarian type stroma and no dysplasia. The diagnosis of cystadenoma is rare, even more so when not communicating with the bile duct. It requires a high degree of suspicion and a multi-disciplinary approach to consider the diagnosis. Due to malignant potential and recurrence, they should be excised. Consider this unusual diagnosis in females with painless jaundice, elevated LFTs, and solitary cystic lesion.Figure 1Figure 2: H&E; peroxidase stains.