Resident Maimonides Medical Center Brooklyn, New York
Daria Yunina, MD, Daniel J. Waintraub, MD, Richard M. Fazio, MD, Ismail Zahir, MD, Mohammad Hamshow, MD, Kevin Tin, MD, Dmitriy O. Khodorskiy, MD; Maimonides Medical Center, Brooklyn, NY
Introduction: Intraductal papillary neoplasm of the bile duct (IPNB) is a rare variant of bile duct tumor, comprising 9-38% of all bile duct carcinomas. 40-80% of IPNB’s contain a component of invasive carcinoma, therefore, accurate diagnosis is crucial.
Methods: A 68-year-old Asian male with past medical history of hypertension and chronic obstructive pulmonary disease (former tobacco use) was referred for evaluation of progressively worsening right upper quadrant abdominal pain and abnormal imaging. An outpatient magnetic resonance imaging (MRI) revealed marked dilation of left intrahepatic biliary tree with distal duct lesion. Endoscopic ultrasonography (EUS) revealed a 26.3 x 28.3 mm, irregularly-shaped, hyperechoic, homogenous left intrahepatic duct lesion. Fine needle biopsy (FNB) was not performed due to concern for cholangiocarcinoma. Endoscopic retrograde cholangiopancreatography (ERCP) with cholangioscopy revealed a non-obstructing, soft, irregular intraductal mass with papillary projections in distal left intrahepatic system just upstream to the bifurcation. Targeted biopsies were obtained utilizing Boston Scientific SpyBite biopsy forceps. Pathology revealed IPNB with low-grade dysplasia (LGD) with inability to evaluate for invasion. Patient was referred to hepatobiliary surgery and underwent open left hepatectomy. Final pathology confirmed IPNB with LGD, was found to be of oncocytic type and had no evidence of invasion. Immunohistochemical stains were diffusely positive for MUC5AC and MUC6, supporting the diagnosis. The patient recovered well and no further treatment was deemed necessary. Discussion: IPNB’s are typically found in patients aged 50-70 years old from the Far East with a history of hepatolithiasis or clonorchiasis. Most are asymptomatic, however, some patients present with abdominal pain, jaundice or cholangitis. Imaging findings include intraductal masses, which can be multifocal, and varying patterns of bile duct dilation. Majority of IPNB’s are found in the left ductal system. EUS and intraductal ultrasound can assess the depth of invasion and lymphadenopathy. Cholangioscopy can confirm histology and extent of disease. Pathologic diagnosis does not always reflect the maximum degree of atypia because IPNB’s are composed of varying degrees of cytoarchitectural atypia. Diagnosis and staging are imperative as surgical resection can be curative. The prognosis is better than that of cholangiocarcinoma, with 5-year postoperative survival rates approaching 70%.
EUS showing left liver lobe lesion
Cholangiogram (shown on the left) and Cholangioscopy (shown on the right)
Oncocytic papillary proliferation with gastric and goblet type cells in 10x (shown on the left) and 40x (shown on the right)
Disclosures: Daria Yunina indicated no relevant financial relationships. Daniel Waintraub indicated no relevant financial relationships. Richard Fazio indicated no relevant financial relationships. Ismail Zahir indicated no relevant financial relationships. Mohammad Hamshow indicated no relevant financial relationships. Kevin Tin indicated no relevant financial relationships. Dmitriy Khodorskiy indicated no relevant financial relationships.