Intraductal Papillary Neoplasms of the Bile Duct: Clinical Case Insights and Literature Review

Abstract

Background: Intraductal papillary neoplasms of the bile duct (IPNB) are rare precancerous lesions with implications for the development of cholangiocarcinoma (CCA). Recognizing IPNB and managing its recurrence pose challenges in clinical practice. We present two cases. Case 1: a 60-year-old man presented with an 8 × 8 × 9 cm hepatic cyst initially suspected to be a hydatid cyst. Histology post-resection revealed an IPNB with foci of adenocarcinoma. Despite negative oncologic margins, recurrence occurred eight years later as an rT2N0 lesion. Surgical resection was performed without adjuvant chemotherapy, resulting in the patient's survival at 48 months post-surgery. Case 2: a 28-year-old female with cognitive impairment was admitted with pulmonary embolism and a liver lesion diagnosed as a simple cyst. Subsequent evaluation revealed adenocarcinoma with local metastases, extensive vascular involvement, and thrombosis. Despite aggressive management, including thrombectomy and chemotherapy, the patient's condition deteriorated, leading to hepatic failure and eventual demise.

Conclusion: IPNB represents a rare premalignant subtype with a propensity for progression to CCA. R0 surgical resection typically offers favorable oncological outcomes with a minimal recurrence risk. Surgical intervention for localized resectable recurrence is both safe and feasible. International registries tracking IPNB recurrence are essential for advancing understanding and optimizing diagnosis, management, and treatment strategies.

Keywords: cholangiocarcinoma; intraductal papillary neoplasm of the bile duct; liver resection.

Figure 1

(a,b): MRI T2 axial (a) and coronal (b) images showing a large cystic mass involving the IV hepatic segment. An irregular-shaped mass is present on the posterior boundary of the cyst (arrows). The portal vein appears dislocated downward by the cyst.

Figure 2

MR cholangiography showing a large, well-defined mass in close proximity to the biliary tree. The mass appears to cause displacement of the biliary ducts, suggesting a potential compressive effect rather than direct invasion.

Figure 3

EE 100× cystic adenoma. Lining epithelium was composed of cuboidal and columnar cells, many of which had an abundant eosinophilic cytoplasm producing an oncocytic appearance.

Figure 4

EE 400× epithelial cells showed varying degrees of dysplasia, in some places high-grade.

Figure 5

CT axial and coronal planes on parenchymal window showing a multicystic lobulated mass involving the hepatic hilum. After injection of iodinated contrast, the mass appears hypo-vascular, with enhancing margins and internal septations (a,b). PET axial and coronal images show increased uptake of ¹⁸[F]-fluorodeoxyglucose in the multicystic lobulated mass involving the hepatic hilum suspicious for recurrences of IPBN (c,d).

Figure 6

MRI T2 axial (a,b), T2 coronal (c), and MR cholangiography (d) images showing a multicystic lobulated mass with internal septations involving the hepatic hilum and causing dilation of the intrahepatic biliary ducts.

Figure 7

(a–c): MR images with T2 sequence in axial and coronal planes revealing a 10 cm diameter solid cystic lesion with vegetations along its walls. The images illustrate marked dilation of the intrahepatic bile ducts associated with the lesion. Additionally, diffuse signal alteration and centimetric nodules within the surrounding hepatic parenchyma suggest widespread disease localization; (d,e): axial T1-weighted images, post-intravenous administration of paramagnetic contrast agent, showcasing enhancement of the solid components of the lesion, internal vegetations, and the surrounding parenchyma infiltrated by the disease.

Figure 8

Laparoscopic view of the lesion.

Figure 9

Histological characteristics of the tumor. Notice the papillary features with pools of mucin and a cystic/pseudocystic appearance. The neoplasm exhibits strong acute inflammation and areas of necrosis. Based on its immunohistochemical profile, the diagnosis of IPNB was confirmed.