Clinicopathologic review of 58 patients with biliary papillomatosis

Abstract

BACKGROUND Biliary papillomatosis (BP) is a rare disease that is characterized by multiple numerous papillary adenomas in the biliary tree. The clinical features and outcome, however, are not well known. The authors retrospectively analyzed their clinicopathologic features and long‐term follow‐up results. METHODS Between March 1995 and January 2003, 58 patients were diagnosed with BP by cholangioscopic and histologic findings at a tertiary referral center, Asan Medical Center (University of Ulsan College of Medicine, Seoul, Korea). The authors retrospectively reviewed the medical records to obtain demographic, radiologic, cholangioscopic, and pathologic data. RESULTS The common clinical manifestations at the presentation of patients were repeated episodes of abdominal pain, jaundice, and acute cholangitis. Acute cholangitis was more common in patients with mucin‐hypersecreting BP (MBP), whereas patients with nonmucin‐producing BP (NMBP) were more asymptomatic (P < 0.05). Papillary adenocarcinoma and mucinous carcinoma were detected in 48 patients (83%) with papillary adenomas. Overall survival rates of NMBP and MBP were 89% and 69% at 1 year, 57% and 37% at 3 years, and 52% and 19% at 5 years, respectively. The mean survival period of NMBP and MBP was 52.27 ± 6.72 months and 30.84 ± 8.36 months, respectively. CONCLUSIONS BP should be regarded as a premalignant disease with high malignant potential. The pathogenesis of progression from benign to malignant disease may follow the adenomacarcinoma sequence. Although clinical presentations were somewhat different for patients with NMBP and MBP, the long‐term survival rate was similar. Cancer 2004;100:783–93. © 2004 American Cancer Society.

Biliary cystic tumors with bile duct communication: a cystic variant of intraductal papillary neoplasm of the bile duct

“…From the view point of papillary proliferation, discrimination between IPN-B and biliary papillomatosis or cholangiocarcinoma of intraductal growth-type might be a problem. [29][30][31][32][33] We now speculate that most of biliary papillomatosis and cholangiocarcinoma of intraductal growth-type can be regarded as IPN-B, although a detailed clinicopathological study using a large numbers of cases is mandatory to conclude this issue. Biliary tumors with abundant mucin production have been called mucin-hypersecreting bile duct tumors.…”

GNAS Codon 201 mutations are uncommon in Intraductal Papillary Neoplasms of the Bile Duct

“…2 These neoplasms can arise as either solitary or multifocal lesions within the biliary tree; in the latter scenario they are termed in classical parlance as 'biliary papillomatosis' . [3][4][5][6] Intraductal papillary neoplasms of the bile duct show considerable morphologic similarities to intraductal papillary mucinous neoplasms (IPMNs) of the pancreas. Both entities present with a prominent intraductal papillary component within larger-diameter ducts, both are mucinproducing, both harbour varying grades of dysplasia in the lining epithelium, and both exhibit distinct patterns of differentiation, such as intestinal and pancreatobiliary manifestations.…”

“…The accompanying jaundice arises as a result of the physical obstruction of bile outflow by the neoplasm itself or the viscous inspissated mucin. 5 There are no evident risk factors for IPNBs, although some studies have reported an association with longstanding biliary inflammation. 5,8 Because of the underlying risk for malignant transformation to invasive adenocarcinomas (cholangiocarcinomas) 5 and the obstructive nature of the lesions, resection with clear margins is the therapy of choice for IPNBs.…”

“…5 There are no evident risk factors for IPNBs, although some studies have reported an association with longstanding biliary inflammation. 5,8 Because of the underlying risk for malignant transformation to invasive adenocarcinomas (cholangiocarcinomas) 5 and the obstructive nature of the lesions, resection with clear margins is the therapy of choice for IPNBs. Unfortunately, multifocal IPNBs may recur even after apparently curative resection and, in rare cases with diffuse intrahepatic disease, orthotopic liver transplantation may be considered.…”

A novel approach to biliary tract pathology based on similarities to pancreatic counterparts: Is the biliary tract an incomplete pancreas?

“…Among them, biliary papilloma is a rare neoplasm composed of papillary proliferation of biliary epithelium with delicate fibrovascular stalks, and multiple occurring biliary papillomas are known as biliary papillomatosis. 43,44 Billiary papilloma and biliary papillomatosis (BPs) are known to progress to invasive carcinoma. Some CCs also show mainly papillary proliferation in the bile duct lumen and also shows invasive lesions, and those cases are classified as the papillary type of bile duct carcinoma or intraductal growth-type of ICC (papillary CC).…”