Daniel J. Ellis, MD1, Janak Shah, MD2, John A. Evans, MD2, Ricardo V. Romero, MD2, Abdul H. El Chafic, MD2 1Ochsner Medical Center, Dallas, TX; 2Ochsner Medical Center, New Orleans, LA
Introduction: Mirizzi syndrome is the extrinsic compression of the common hepatic duct due to cholelithiasis. Although uncommon (5.7% of cholecystectomy for cholelithiasis), a substantial proportion of Mirizzi patients have underlying gallbladder cancer (5 – 28%) – often undiagnosed prior to cholecystectomy. We report a case of malignancy presenting as Mirizzi syndrome diagnosed endoscopically prior to cholecystectomy, which facilitated multidisciplinary oncologic workup and therapy.
Case Description/Methods: A 56-year-old female presented with 1 month of abdominal pain and jaundice. Magnetic resonance cholangiopancreatography identified intra-hepatic biliary dilation and a cystic duct stone causing compression on the common bile duct (CBD, figure a). The patient clinically improved, she declined laparoscopic cholecystectomy and was discharged. She re-presented 1-week later with worsening pain and further elevation of liver tests. CT showed a calcified stone near the site of CBD transition (figure b). Labs were notable for elevated CA 19-9 (11,000). She underwent endoscopic evaluation. Endoscopic ultrasound (EUS) was limited due to gallstone shadowing but identified a soft tissue density arising from the bile duct adjacent to the stone (figure c). EUS sampling was not performed due to potential liver transplant candidacy if malignancy was confirmed. ERCP identified a proximal CBD stricture characterized by nodularity and neovascularization on cholangioscopy (figure d, e). Tissue obtained through cytology brushing was negative, whereas cholangioscopy-guided biopsies were positive for adenocarcinoma. She underwent planned oncologic resection, but this was deferred after staging laparoscopy identified metastatic lesions on the liver surface. She was referred for chemotherapy.
Discussion: Gallbladder malignancy may masquerade as Mirrizi syndrome, and it is important to maintain suspicion during diagnostic evaluation. Although benign obstruction, including Mirizzi, may elevate CA 19-9, significantly elevated levels are concerning for malignancy. In these cases, cholangioscopy facilitates direct visualization, identification of high-risk features (e.g. neovascularization), and allows for direct biopsies with high tissue sampling yield. It is important to differentiate benign Mirizzi syndrome from gallbladder cancer as therapeutic approaches greatly differ and a multidisciplinary management is beneficial.
Figure: a) MRI demonstrating intra-hepatic biliary and proximal common bile duct (CBD) dilatation with abrupt tapering (arrow) at the level of the cystic duct due to extrinsic compression from a low signal structure consistent with gallstone (arrowhead). b) CT showing biliary dilatation with abrupt transition at site of partially calcified gallstone (arrow). c) EUS demonstrating common hepatic (CHD) dilatation and adjacent gallstone. Small hypoechoic lesion adjacent to common hepatic duct (asterisk) d) Cholangiogram with CBD stricture (arrow). e) Cholangioscopy image at the level of the biliary stricture with mucosal scaring, inflammation and neovascularization.
Disclosures:
Daniel Ellis indicated no relevant financial relationships.
Janak Shah indicated no relevant financial relationships.
John Evans indicated no relevant financial relationships.
Ricardo Romero indicated no relevant financial relationships.
Abdul El Chafic indicated no relevant financial relationships.
Daniel J. Ellis, MD1, Janak Shah, MD2, John A. Evans, MD2, Ricardo V. Romero, MD2, Abdul H. El Chafic, MD2. P2196 - Malignancy Masquerading as Mirizzi Syndrome, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.
.jpg)