33-year-old-male-patient:
-
- Presented with jaundice
- laboratory tests revealed elevated levels of serum alkaline phosphatase (ALP), gamma-glutamyl transferase (GGT), total bilirubin, and direct bilirubin
What do you see?
-
- A lobulated mass within the bile ducts (shown in red areas)
- Dilatation of intrahepatic bile ducts
-
- A 3.5-cm-lobulated mass within the bile ducts (red arrows) with
upstream and downstream dilatation of bile ducts
- A 3.5-cm-lobulated mass within the bile ducts (red arrows) with
What is your diagnosis?
-
- Intraductal papillary neoplasm of the bile duct (IPNB) with invasive carcinoma
- In our case, the patient underwent surgery and the diagnosis was histopathologically confirmed
Teaching points
-
- IPNBs are premalignant biliary epithelial tumors.
- IPNBs originate from and communicate with the biliary tree and can occur anywhere along the biliary tree.
- 30-40% of IPNBs exhibit mucin hypersecretion.
- When symptomatic, patients present with recurrent abdominal pain, cholangitis, and jaundice.
- Risk factors for IPNBs include hepatolithiasis, clonorchiasis, primary sclerosing cholangitis, biliary tree malformations, choledochal cysts, and familial adenomatous polyposis.
- IPNB is analogous to the intraductal papillary mucinous neoplasm of the pancreas (IPMN).
- Imaging findings of IPNB include:
-
- Intraductal mass with upstream and downstream dilatation
- Complex cystic mass with biliary ductal dilatation
- Ductal dilatation without a mass, with associated parenchymal atrophy
- Thread sign at MRCP (linear and curved filling defects due to mucin)
-
- IPNBs may be associated with invasive carcinoma (27-94%)
- Imaging findings that suggest invasive carcinoma at MRI include
-
- A visible intraductal mass
- Tumor size greater than 2.5 cm
- Tumor multiplicity
- Ductal wall thickening
- Adjacent hepatic invasion
-
- Early surgical intervention is the key management for IPNB.
- All patients with IPNB should be considered for treatment, given the high potential for malignancy and for recurrent cholangitis and obstructive jaundice in nonmalignant cases
Klatskin tumor