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


CONCLUSIONS
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.
Liver and Pancreas Unit, Department of Surgery, University of Ottawa, Ottawa, ON, Canada.
Sean
S
Bennett
Department of Pathology and Laboratory Medicine, University of Ottawa, Ottawa, ON, Canada.
E Celia
EC
Marginean
Liver and Pancreas Unit, Department of Surgery, University of Ottawa, Ottawa, ON, Canada.
Melanie
M
Paquin-Gobeil
Department of Pathology and Laboratory Medicine, University of Ottawa, Ottawa, ON, Canada.
Jason
J
Wasserman
Liver and Pancreas Unit, Department of Surgery, University of Ottawa, Ottawa, ON, Canada.
Joel
J
Weaver
Liver and Pancreas Unit, Department of Surgery, University of Ottawa, Ottawa, ON, Canada.
Richard
R
Mimeault
Liver and Pancreas Unit, Department of Surgery, University of Ottawa, Ottawa, ON, Canada.
Fady K
FK
Balaa
Liver and Pancreas Unit, Department of Surgery, University of Ottawa, Ottawa, ON, Canada.|Clinical Epidemiology Program, Ottawa Hospital Research Institute, Ottawa, ON, Canada.
Guillaume
G
Martel
D000368:Aged; D001650:Bile Duct Neoplasms / Q000378:metabolism / Q000401:mortality / Q000473:pathology*; D001653:Bile Ducts, Intrahepatic / Q000473:pathology*; D014408:Biomarkers, Tumor / Q000378:metabolism; D002291:Carcinoma, Papillary / Q000378:metabolism / Q000401:mortality / Q000473:pathology*; D005260:Female; D005706:Gallbladder Neoplasms / Q000378:metabolism / Q000401:mortality / Q000473:pathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D018396:Mucin-1 / Q000378:metabolism; D009864:Ontario / Q000453:epidemiology; D012189:Retrospective Studies; D015996:Survival Rate / Q000639:trends
D014408:Biomarkers, Tumor; D018396:Mucin-1