Non-MalIg(G4)nant Biliary Obstruction: When the Pill Is Mightier than the Knife

A 48-year-old man was initially evaluated in a community hospital with jaundice and elevated liver enzymes (bilirubin 9.1 mg/dL and alkaline phosphatase of 682 U/L). Intrahepatic ductal dilatation had been diagnosed with computed tomography (CT); endoscopic retrograde cholangiopancreatography (ERCP) confirmed this and documented a common bile duct stricture which was stented. Pathology from the brushings indicated cellular atypia but no clear evidence of malignancy. He developed post-procedural pancreatitis and a pancreatic pseudocyst that required prolonged drainage, delaying for several months further evaluation of his common bile duct stricture. Once the pancreatic inflammation had subsided, diagnostic laparoscopy was considered in order to further characterize the etiology of his stricture; however, the operation was aborted as it was felt that the patient had extensive cholangiocarcinoma. He was ultimately discharged but readmitted for persistent jaundice. Mirrizi’s syndrome was diagnosed on the basis of an exploratory laparotomy for which he underwent a cholecystectomy and choledochogastrostomy for biliary drainage. The pathology from the bile duct at the time of surgery did not show malignancy. Postoperatively, the patient struggled with a 40-lb weight loss and multiple episodes of repeated jaundice due to an anastomotic stricture. This problem required several ERCP procedures and stent exchanges over the course of 1 year. His final imaging prior to transfer was an MRCP demonstrating presence of the choledochogastrostomy, bilateral intrahepatic ductal dilation (Left[Right), and considerable atrophy of the left lobe of the liver (Fig. 1). After transfer of care to Stanford, the patient was sent for a percutaneous transhepatic biliary drain (PTBD) to relieve his biliary obstruction (as previous ERCPs had been unsuccessful). A cholangiogram demonstrated a high-grade stricture extending from the anastomosis high into the biliary tree, though a biopsy again did not show malignancy. His CA 19–9 was elevated to 1,106 U/mL, and a new liver triphasic CT scan demonstrated worrisome findings for cholangiocarcinoma. A hypoattenuating mass was present at the confluence of the right and left bile ducts with persistent intrahepatic biliary dilation and chronic left lobe atrophy (Fig. 2). Although the mass, if malignant, was unresectable given involvement of the main hepatic artery, it was felt that his previous workup for malignancy had been inconclusive and a tissue diagnosis would be required in order to initiate chemotherapy. A CT-guided biopsy of the mass was negative for malignancy, only yielding atypical lymphoid cells. This was followed by endoscopic ultrasound and FNA, where the hypoechoic area at the hilum was visualized but again did not yield malignant cells, but rather a cluster of heterogeneous T and B cells without other abnormalities. The patient underwent ERCP and Spyglass choledochoscopy twice through the choledochogastrostomy; the anastomotic stricture was traversed with multiple biopsies at the level of the stenosis were also negative for malignancy (Fig. 3). About a month after his initial PTBD, the patient had recurrent jaundice and M. M. Dua M. Qadan B. C. Visser (&) Division of Surgical Oncology, Department of Surgery, Stanford University Medical Center, 300 Pasteur Drive, Suite H3680C, Stanford, CA 94305, USA e-mail: bvisser@stanford.edu