Endoscopic treatment of a large intraductal papillary mucinous neoplasm of the bile duct: tips and tricks learned during multiple treatments of a difficult case

Intraductal papillary mucinous neoplasm of the bile duct (IPMN-B) is an epithelial tumor recognized as a precursor to cholangiocarcinoma, and it has a higher incidence in Eastern countries. IPMN-B typically presents as a papillary or villous mass within the biliary lumen with high-grade production of mucin, often resulting in obstructive jaundice and/or recurrent episodes of cholangitis. Surgical resection remains the treatment of choice, though several palliative endoscopic approaches have been reported.^{,  ,} 

A 71-year-old woman with multiple medical comorbidities presented to this institution with obstructive jaundice and ascending cholangitis. She underwent multiple ERCPs notable for viscous mucin occluding the extrahepatic biliary system (Fig. 1). EUS and ERCP revealed an obstructing, intraluminal mass with no signs of lymphadenopathy or metastatic disease (Fig. 2). Peroral cholangioscopy revealed the intraluminal tumor with its typical papillary appearance extending from the bifurcation to the distal common hepatic duct (Fig. 3) with biopsies confirming IPMN-B with low-grade dysplasia. Because of the highly viscous nature of the tumor mucin secretion, she experienced repeated hospitalizations owing to recurrent episodes of obstructive jaundice and cholangitis despite stenting. After a multidisciplinary evaluation, the patient was determined to be a poor surgical candidate, and the gastroenterology team planned an aggressive approach with intraductal EMR, with thermal tumor ablation as an alternative back-up strategy for the purposes of palliation and symptom control (Video 1, available online at www.giejournal.org).

During the endoscopy, mucin clearance was accomplished mechanically by repeatedly sweeping with a biliary extraction balloon while infusing 10% acetylcysteine though the contrast port. The ampullary segment of the common bile duct was then dilated to 15 mm using a controlled radial expansion balloon and a narrow-caliber endoscope (TJF-Q190V, GIF XP190N; Olympus Medical Inc, Center Valley, Penn, USA). The endoscopes were introduced into the biliary system through a rigidizing overtube (Pathfinder Endoscope Overtube; Neptune Medical, Burlingame, Calif, USA), which was necessary for endoscope stabilization in the duodenum allowing one-to-one advancement into the biliary system. Once the endoscope was stable and the IPMN-B lesion was well-visualized in the common hepatic duct (Fig. 4), the process of EMR was initiated using a lifting gel (Orise gel; Boston Scientific, Marlborough, Mass, USA) (Fig. 5). EMR proved to be challenging, in part because of the lack of a true submucosal space in the biliary system limiting our ability to lift the lesion.^, A 13-mm hex snare (Captivator I; Boston Scientific) was then used to resect the lesion, which could only be done in part because of the lack of a true submucosal lift as previously mentioned. Residual tumor was ablated with argon plasma coagulation (APC) (ERBE USA, Marietta, Ga, USA) using sufficiently high wattage at 35 W and 0.8 L/min flow (Fig. 6). Care was taken to frequently remove any remaining mucin, as the mucin acted as a shield against effective APC thermal ablation.

There are several lessons we learned in this case. First, endoscope stabilization in the duodenum is paramount to avoid bending and bowing into the third portion duodenum as the endoscope is advanced into the biliary system. Here, stabilization was achieved using the Neptune Medical rigidizing overtube. Second, EMR in the biliary lumen is challenging because of a lack of a true submucosal space, making lifting the lesion very difficult. Third, ablation with APC is effective if sufficient wattage is used to ablate the thick tumor; however, the mucin must be thoroughly removed as it acts as a shield against the thermal effect of APC. For this, we used a combination of aggressive sweeping of the bile duct with lavage of 10% acetylcysteine, as well as mechanical removal with the snare and cytology brush.

IPMN-B is an epithelial tumor and precursor to cholangiocarcinoma. The treatment of choice is surgical resection, though different endoscopic interventions for palliation of symptoms have been reported. Here we show that the use of a narrow-diameter upper endoscope allows excellent visualization and multiple treatment options to be directed at the lesion. However, duodenal endoscope stabilization is paramount for effective biliary endoscopy, treatment with EMR is challenging because of a lack of a true submucosal layer, and effective APC treatment of the lesion requires sufficient wattage and aggressive mucin clearance.