Our second clinical spotlight features an IPMN case treated by Dr Raj Shah and his team (Drs Nicholas Brown and Joel Camilo) at the University of Colorado.  We recommend that you be on the lookout for a more detailed journal publication over the next few months. (Update: the case is now published formally and can be accessed here.)

A 79-year-old man was admitted with two weeks of fatigue, abdominal discomfort and jaundice. He was an active smoker. Physical exam revealed a frail, debilitated male with jaundice, icterus, and mild tenderness to palpation in the right upper quadrant and epigastrium. CT scan reported a pancreatic head cystic mass with both biliary and pancreatic ductal dilatation. He underwent ERCP and EUS-FNA with plastic stent placement. Cytopathology revealed IPMN without dysplasia. He was referred to the University of Colorado Hospital due to persistent jaundice and failure to thrive despite biliary stenting.  

Contrast-enhanced computed tomography (CT) demonstrated a diffusely dilated main pancreatic duct (16 mm), multiloculated cystic mass (6 cm) in the pancreatic head and uncinate process, evidence of biliary obstruction with intra- and extrahepatic ductal dilation up to 18 mm and moderate narrowing of the portosplenic confluence. Due to poor operative candidacy given functional status and CT findings, palliative options were pursued along with additional tissue sampling to confirm suspected malignancy. Repeat EUS and ERCP at UCH was performed one week after the index procedures at the referring hospital. The ampulla had the typical gaping orifice seen with IPMN but included both the biliary and pancreatic orifice. Cholangiography revealed mucinous filling defects in the lower and mid bile duct with marked dilatation. After forceps biopsy from the pancreatic duct, two overlapping covered self-expanding metal stents with the downstream stent bridging the papilla were placed.  

Cholangiopancreatography revealing mucinous filling defects in both ducts at follow-up ERCP of index biliary RFA session

Cholangiopancreatography revealing mucinous filling defects in both ducts at follow-up ERCP of index biliary RFA session

Histology revealed mucinous epithelium with low-grade dysplasia and EUS-FNA of the pancreatic head cyst revealed IPMN with moderate dysplasia. The patient’s bilirubin improved initially but rose again to 22.6 mg/dL prompting repeat evaluation locally one week later. Repeat CT demonstrated that the previously placed stents had migrated entirely out of the extrahepatic ducts into the rectum which were subsequently passed naturally. He remained too fragile for surgical resection and was sent for consideration of repeat endoscopic therapy. Biliary RFA was pursued after careful discussion with the patient on the potential benefits and risks. He underwent two separate sessions of RFA via ERCP two months apart.   During both procedures the bile duct was explored endoscopically using the Boston Scientific SpyGlass direct visualization system.

Cholangioscopy under fluoroscopic guidance confirmed a villous mass with a fistulous connection between the area of the pancreatic head cyst and the extrahepatic bile duct. Further, given the mucinous filling defects within the bile duct, it was difficult to discern the exact margins of tumor involving the bile duct and thus, cholangioscopy was used to fluoroscopically mark the upper and lower margins of the villous tumor burden.

Fluoroscopic images of the cholangioscope at upper and lower margins of the visualized villous mass

Fluoroscopic images of the cholangioscope at upper and lower margins of the visualized villous mass

During his first RFA, the HabibTM EndoHPB was advanced over a .035” wire and to the upper, mid and lower margins of the tumor as estimated by the fluoroscopic spot films of the cholangioscope position. Three ablations were performed (8W, Effect 8 and 90 seconds each site with one minute “cooling” interval prior to adjustment to an additional site) under fluoroscopy guidance. Three side-by-side plastic stents were placed. Seven weeks later, he had clinically improved with respect to jaundice, appetite, and weight. Cholangiography revealed a subjective improvement in the degree of mucinous filling defects. He underwent a second biliary RFA session that included two overlapping stations (8W, 90 seconds per application) under cholangioscopy and fluoroscopy guidance as described above. Prior stents were partially occluded with mucin and tumor and these were replaced.

Three weeks later, his CA 19-9 had dropped to 142 unit/mL, total bilirubin to 1.3 mg/dL and albumin rose to 3.1 g/dL. Appetite, functional status, and weight improved as well. A repeat CT showed a grossly unchanged cystic mass in the pancreatic head, main PD dilation to 18mm and extrahepatic ductal dilation to 20mm but the surgical team felt that the cystic mass, though it appeared that his entire main duct was involved, had an improved plane with the major vessels for potential resection. Due to this and his improved functional status five weeks after his last RFA session, he underwent a classic Whipple’s surgery with portal vein reconstruction. Intraoperative frozen sections of the bile duct were negative, a margin on the portal vein was negative and the neck margin of the pancreas did not have malignancy, although it did have IPMN. All gross disease at the level of pancreatic head cystic mass was resected. The final surgical pathology showed IPMN with high-grade dysplasia/carcinoma in situ, with the pancreatic neck margin revealing moderate dysplasia.  He is clinically well and at pre-illness functional status 17 months following surgery.  

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AuthorFrancois Poulin