Intrahepatic intraductal papillary cystic neoplasm of the bile duct: A case report

Abstract

Introduction and importance Intraductal papillary neoplasm of the bile duct (IPNB) is a tumour with a very low incidence in the Western world, characterised by a high risk of malignant transformation and unknown prognosis. It is a new entity which was adopted by the WHO in 2010 as a precursor lesion of cholangiocarcinoma. Intrahepatic bile duct is the most common site of origin for IPNB. Case presentation Hereby, we present a case of an asymptomatic 63- year-old man, referred to our department after routine ultrasonography showing a multifocal cystic lesion on the left hepatic lobe. Further screening modalities (CT, MRI abdo) confirmed a complex cystic liver lesion with atypical features. The patient underwent left hepatectomy. Histopathology showed a cystic type intrahepatic IPNB, which was completely resected (R0). The follow up in 2 yrs post-operation showed no signs of recurrence. Clinical discussion The diagnosis and management of IPNB remain challenging. A multimodality imaging approach is essential in order to diagnose IPNB, assess tumour location and extent and plan the optimal treatment strategy. Conclusion Complete surgical resection (R0) with close postoperative follow-up offers long-term survival.

Trick or Treat: An Intrahepatic Intraductal Papillary Neoplasm of the Bile Duct

“…Histologically, it could be subdivided as IPNB with dysplasia (either low grade or high grade) or invasive carcinoma [11]. Approximately 40-80% of the cases present with an underlying invasive carcinoma [5][6][7][8][9][10][11][12].…”

Complex cystic liver lesions: classification, diagnosis, and management

“…In type 4 IPNBs, mucin overproduction leads to increased intraductal pressure and aneurysmal biliary dilation or cystic dilation of peribiliary glands [ 7 , 42 ]. These cystic-type IPNBs present as multilocular cystic masses with a “bunch of grapes” appearance and enhancing mural nodules ( Fig.…”

“…Similarly, MCNs in contrast to cystic-type IPNBs are not accompanied by biliary dilation. Gadolinium-based agents, excreted into the lesion on the hepatobiliary phase, are quite helpful in this regard [ 31 , 42 ]. Apart from MCNs, the differential diagnosis should include complicated hepatic cysts, localized Caroli disease, and more rare cystic hemangiomas, lymphangiomas, hepatic foregut cysts, mesenchymal hematomas or teratomas [ 1 , 31 ].…”

“…The extent of surgical resection depends on the tumor location, the degree of biliary tract involvement and patient suitability. Most patients are sufficiently treated with radical surgical resection, which includes hepatectomy with or without bile duct resection [ 42 , 52 ]. In extreme cases with more aggressive IPNBs and positive resection margins, major hepatectomy or orthotopic liver transplantation, with or without pancreaticoduodenectomy, may be performed.…”

“…In extreme cases with more aggressive IPNBs and positive resection margins, major hepatectomy or orthotopic liver transplantation, with or without pancreaticoduodenectomy, may be performed. IPNBs undergoing curative surgical resection present a significantly better prognosis compared to cholangiocarcinomas, with 5-year disease-free survival as high as 81% [ 31 , 40 , 42 ]. Positive resection margins, positive lymph nodes, tumor invasion ≥5 mm and invasive component ≥10% are all associated with a poorer prognosis [ 31 ].…”