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. 2015 Sep;17(9):811-8.
doi: 10.1111/hpb.12460.

Clinical and pathological features of intraductal papillary neoplasm of the biliary tract and gallbladder

Affiliations

Clinical and pathological features of intraductal papillary neoplasm of the biliary tract and gallbladder

Sean Bennett et al. HPB (Oxford). 2015 Sep.

Abstract

Background: Intraductal papillary neoplasms of the biliary tract (IPNB) and intracholecystic papillary neoplasms (ICPN) are rare tumours characterized by intraluminal papillary growth that can be associated with invasive carcinoma. Their natural history remains poorly understood. This study examines clinicopathological features and outcomes.

Methods: Patients who underwent surgery for IPNB/ICPN (2008-2014) were identified. Descriptive statistics and survival data were generated.

Results: Of 23 patients with IPNB/ICPN, 10 were male, and the mean age was 68 years. The most common presentations were abdominal pain (n = 10) and jaundice (n = 9). Tumour locations were: intrahepatic (n = 5), hilar (n = 3), the extrahepatic bile duct (n = 8) and the gallbladder (n = 7). Invasive cancer was found in 20/23 patients. Epithelial subtypes included pancreatobiliary (n = 15), intestinal (n = 7) and gastric (n = 1). The median follow-up was 30 months. The 5-year overall (OS) and disease-free survivals (DFS) were 51% and 57%, respectively. Decreased OS (P = 0.09) and DFS (P = 0.05) were seen in patients with tumours expressing MUC1 on immunohistochemistry (IHC).

Conclusion: IPNB/ICPN are rare precursor lesions that can affect the entire biliary epithelium. At pathology, the majority of patients have invasive carcinoma, thus warranting a radical resection. Patients with tumours expressing MUC1 appear to have worse OS and DFSs.

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Figures

Figure 1
Figure 1
(a) Liver left hepatectomy surgical resection specimen, showing a large papillary tumour filling the dilated intrahepatic bile ducts (thick arrows). (b) Tumour depicted in (a), showing the intraductal papillary neoplasm (IPNB) that fills the bile duct lumen and spreads along the biliary tree (thick arrows). An area of invasive adenocarcinoma into the liver parenchyma is noted, tubular type, poorly differentiated (thin arrow). Hematoxylin and eosin (H&E) stain, 2× magnification. (c) IPNB, common bile duct (thick arrows). Invasive adenocarcinoma is present, tubular type, invading the duct wall into the periductal adipose tissue (thin arrow). H&E stain, 2× magnification. (d) IPNB, pancreatico-billiary type. Tumour cells resemble biliary or pancreatic epithelium, are composed of columnar cells or low cuboidal cells with eosinophilic cytoplasm and round nuclei. H&E stain (200x). (e) IPNB, intestinal type. Tumour cells resemble intestinal adenoma or adenocarcinoma and are characterized by stratified tall columnar cells with some goblet cells. H&E stain (200x). (f) IPNB, gastric type. Tumour cells resemble gastric epithelium and are composed of columnar cells with abundant intracytoplasmic mucin. H&E stain (200x)
Figure 2
Figure 2
Kaplan–Meier survival curves for (a) overall survival, (b) disease-free survival, (c) overall survival with/without MUC1, and (d) disease-free survival with/without MUC1

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