Intraductal papillary neoplasm of the biliary tract (IPNB) is a rare clinical entity
that may present with mucobilia and has a high risk of malignant transformation to
cholangiocarcinoma. These papillary neoplasms are characterized by superficial spread,
dilated bile ducts, and multifocal distribution. Surgery is the treatment of choice.
Endoscopic therapy may offer palliation.
An 86-year-old woman presented to the emergency department with obstructive jaundice
and sepsis due to ascending cholangitis. Her initial MRCP revealed dilated bile ducts
without any mass or stone in the ductal system (Fig. 1A). ERCP revealed soft filling
defects in the common bile duct consistent with mucobilia (Fig. 1B). The adult gastroscope
was introduced into the common bile duct via fluoroscopic guidance (Fig. 1C) and into
the common hepatic duct (Fig. 1D). No mass lesions were seen following duct clearance.
Unfortunately, she was not a surgical candidate because of her multiple medical comorbidities.
Despite endoscopic therapy, the patient experienced multiple episodes of recurrent
ascending cholangitis resulting from mucus impaction. On follow-up endoscopic evaluation,
per-oral cholangioscopy (POC) with a GIF 180 adult gastroscope (Olympus, Center Valley,
Pa, USA) demonstrated mucobilia, dilatated bile ducts, and nodularities in the cystic
duct (Fig. 1E). Analysis of biopsy specimens revealed an intraductal papillary neoplasm
with low-grade dysplasia from the nodularities of the cystic duct (Fig. 1F). Endoscopic
treatment modalities were discussed with the patient to prevent recurrent mucobilia
and cholangitis. Argon plasma coagulation (APC) was chosen (Video 1, available online
at www.VideoGIE.org).
Figure 1
A, MRCP view showing dilated common bile and hepatic ducts. B, ERCP view showing mucobilia
as a filling defect that was aggressively cleared (left). No mass lesion was seen
on occlusion cholangiogram following clearance (right). C, Fluoroscopic image of adult
gastroscope in the common bile duct. D, Endoscopic view showing the common hepatic
duct and bifurcations. E, Endoscopic view showing nodularities of the cystic duct
(left) with narrow-band imaging (right). F, Staining of biopsy specimen showing intraductal
papillary neoplasm of the bile duct, gastric type. Mucinous epithelium-forming papillary
structures also seen here (left, H&E, orig. mag. ×10; right, H&E, orig. mag. ×20).
G, Post-argon plasma coagulation treatment of nodularities.
An APC probe was set at 15 to 25 watts, in forced mode, with effect 1, and 0.8 to
1.2 L/min. Visible cystic duct nodularities received treatment with APC (Fig. 1G).
Pulses were followed by suction to reduce argon gas distention. At a 14-week follow-up
visit, ERCP and POC showed significant reduction in mucobilia, and a scar was seen
at the sites of previous treatment. The remaining areas of nodularity were treated
with APC. The patient has done well after therapy without recurrent symptoms.
IPNB is a rare disease entity with a high risk of malignant transformation, and, as
such, surgical resection is the treatment of choice. However, nonsurgical candidates
may benefit from minimally invasive endoscopic therapies. Nonsurgical management of
biliary neoplasms with the use of APC is a well-tolerated and safe procedure. Endoscopic
therapeutic strategies may improve morbidity and patient survival associated with
IPNB.
Disclosure
All authors disclosed no financial relationships relevant to this publication.