Intraductal Papillary Neoplasm of the Bile Duct: A Diagnostic Challenge for Optimal Treatment (original) (raw)
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Intrahepatic Intraductal Papillary Cystic Neoplasm of the Bile Duct: A Case Report
HPB, 2021
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.
Clinicopathological features of the intraductal papillary neoplasms of the intrahepatic bile duct
Korean Journal of Hepato-Biliary-Pancreatic Surgery, 2012
Backgrounds/Aims: This study is intended to investigate the clinicopathological features of the intraductal papillary neoplasms of the intrahepatic bile duct (IPNB), especially focused on malignant changes. Methods: From the institutional database of liver resection cases (Asan Medical Center, University of Ulsan College of Medicine), 18 patients who met the definition of IPNB were selected. They had undergone liver resection between February 2002 and October 2006; thus, the follow-up period was more than 5 years. Results: Of the 18 patients, 11 patients were male. Their mean age was 61.3±6.7 years. There were no differences between the non-malignant and malignant lesions, in the comparison of the CEA levels (5.6±2.7 vs.12.6±31.1 ng/ml, p=0.439) and the CA19-9 levels (29.2±34.7 vs.31.9±30.2 ng/ml, p=0.871). The common radiologic findings were: intraductal growing mass in 10; bile duct dilatation in 6; and saccular duct dilatation in 2. Left and right hepatectomies were performed in 15 and 3, respectively. Five patients showed benign lesions of IPNB, and 13 patients revealed malignant lesions of intraductal papillary adnocarcinoma or cholangiocarcinoma. All 4 patients with benign lesions survived for a mean period of 53 months without recurrence. In 13 patients with the malignant lesions, 1-year, 3-year, and 5-year survival rates were 100%, 84.6%, and 59.2%, respectively. Conclusions: We concluded that intrahepatic IPNB is a rare type of biliary neoplasm which includes a histological spectrum, ranging from benign disease to invasive malignancy. The long-term survival was anticipated after complete curative resection.
Intraductal papillary mucinous tumor of bile ducts radiologic and pathologic features: a case report
Cases Journal, 2008
We report a case of a 67-year-old Caucasian man with right upper quadrant abdominal pain. He underwent radiologic investigations that revealed a solid, focal mass, at the V hepatic segment. Because a definitive diagnosis, based on imaging appearance of the lesion, was impossible in our case, we performed a hystopathological investigation but the biopsies were inconclusive. So, the definitive diagnosis of intraductal papillary mucinous tumor of bile ducts was made on surgical resected material. Intraductal papillary neoplasm of the liver (IPNL) is a recently recognized entity which closely resembles an intraductal papillary mucinous tumor (IPMT) of the pancreas.
Clinical Features of Intraductal Papillary Neoplasm of the Bile Duct Report of 3 Cases Case Reports
Jikeikai medical journal, 2016
Intraductal papillary neoplasm of the bile duct (IPNB) is a recently updated concept that was classified by the World Health Organization classification scheme revised in 2010 as a cystic tumor of the liver with mucinous epithelium. An IPNB is characterized by the absence of ovarianlike stroma and the presence of bile duct communication (BDC), unlike a mucinous cystic neoplasm. An IPNB can sometimes be accompanied by marked dilatation of the bile duct due to hypersecreted mucus. However, the definition of IPNB remains controversial because of clinicopathological differences from intraductal papillary mucinous neoplasm (IPMN). We describe the clinicopathological features of 3 cases of IPNB.
Cancer Reports and Reviews
Background: Intraductal papillary neoplasm of the bile duct (IPNB) is a relatively rare bile duct neoplasm in the multistep carcinogenesis pathway towards cholangiocarcinoma. The diagnosis of IPNB is challenging, and its malignant potential means that its prognosis is related to the performance of R0 resection. We experienced a patient with IPNB detected during close examination of colonic disease and subsequently treated surgically. Case presentation: A 54-year-old man presented to an outside hospital with abdominal pain. Enhanced computed tomography showed wall thickening in the ascending colon and a cystic lesion close to the posterior segmental branch of the bile duct in the liver. Colonoscopy showed diverticulitis and severe stenosis in the ascending colon, with no finding of malignancy. Differential diagnosis of the liver tumor was IPNB, mucinous cystic neoplasm, or metastatic tumor from the colonic disease. The patient underwent hepatectomy and simultaneous right hemicolectomy for severe diverticulitis. Histopathological examination of the liver revealed IPNB with an associated invasive carcinoma. Conclusions: We report a relatively rare case of IPNB. Although preoperative diagnosis of IPNB remains challenging, complete resection is important for a favorable prognosis.
Modern Pathology, 2011
Mucinous cystic neoplasm of the liver has been a controversial entity, in particular, regarding differentiation from intraductal papillary neoplasm of the bile duct. In this study, we compared the characteristics of hepatic mucinous cystic neoplasms with ovarian-like stroma (n ¼ 29) to those of cyst-forming intraductal papillary neoplasms of the bile duct (n ¼ 12). Radiological or macroscopic appearance, histological grade of malignancy, and postoperative clinical course were recorded. Immunohistochemistry for biliary or gastrointestinal markers was performed to characterize cell phenotypes. The patients with hepatic mucinous cystic neoplasm were all female and ranged in age from 21 to 67 years, which was significantly younger than that in the patients with biliary intraductal papillary neoplasm. Eighteen mucinous cystic neoplasms (76%) were located in the left lobe, with 13 (54%) in segment IV. Mucinous cystic neoplasms were significantly larger than intraductal papillary neoplasms (median diameter: 110 vs 50 mm, P ¼ 0.008). In contrast to intraductal papillary neoplasms that were all histologically malignant, 26 mucinous cystic neoplasms (90%) were adenomas, 2 (7%) were borderline malignant, and 1 (3%) was a carcinoma in situ. Benign mucinous cystadenomas had the pure biliary immunophenotype, whereas gastrointestinal markers including cytokeratin 20 and mucin core proteins 2, 5AC, and 6 were more frequently expressed in borderline or malignant mucinous cystic neoplasms and biliary intraductal papillary neoplasms. There was no mortality in the patients with mucinous cystic neoplasm, whereas one patient with intraductal papillary neoplasm died of cancer. In conclusion, hepatic mucinous cystic neoplasms and biliary intraductal papillary neoplasms have different clinicopathological characteristics as evidenced by differences in the age and gender of patients, macroscopic appearance, immunophenotypes, and grades of malignancy.
Endoscopic diagnosis of intraductal papillary mucinous neoplasm of the bile duct
Journal of hepato-biliary-pancreatic sciences, 2010
Intraductal papillary mucinous neoplasm of the bile duct (IPMN-B) is considered an uncommon tumor. The purpose of this study was to evaluate the diagnostic utility of endoscopic cholangiography (ERC) with subsequent peroral cholangioscopy (POCS) and/or intraductal ultrasonography (IDUS) for this tumor. From December 1991 to November 2006, a retrospective analysis was made of eight patients with IPMN-B. Their clinical features and the endoscopic diagnostic strategy for POCS and IDUS were reviewed. In all the patients, ERC failed to show papillary tumors, due to coexisting mucin or biliary sludge. POCS was carried out after ERC and it showed the presence and locations of papillary tumors in all patients, except for one with a tumor in the peripheral intrahepatic bile duct (B3). IDUS was performed in seven of the eight patients; in five of these patients, intraductal protruding tumors were clearly visualized, whereas flat tumors were not identified in the remaining two patients. In one...
BMC gastroenterology, 2015
Intrahepatic biliary mucinous cystic neoplasms are rare hepatic tumors and account for less than 5% of intrahepatic cystic lesions. Accurate preoperative diagnosis is difficult and the outcome differs among various treatment modalities.The aim of this study is to investigate the clinico-radiological characteristics of intrahepatic biliary mucinous cystic neoplasms and to establish eligible diagnostic and treatment suggestions. Nineteen patients with intrahepatic biliary cystadenomas and two patients with biliary cystadenocarcinomas were retrospectively reviewed. Their clinico-radiological variables and survival outcome were analyzed. Of the 19 patients with biliary cystadenoma, 16 (84.2 %) were female. 11 (57.9 %) patients had symptoms before operation with the most common presenting symptom being abdominal pain. Among the patients with available data, serum and cystic fluid CA 19-9 levels were invariably elevated and the CA 19-9 level in the cystic fluid was significantly higher th...
Modern Pathology, 2006
Biliary cystic tumors, which are also called biliary cystadenoma and cystadenocarcinoma, are thought to be a heterogeneous disease entity, and some of them are known to show a luminal communication to the bile duct. In this study, we examined the clinicopathological features of nine cases of biliary cystic tumors with bile duct communication. They were composed of five males and four females with an average age of 67 years (52-84 years). They were multilocular (eight cases) or unilocular (one case), and all cases contained mucinous fluid. A direct luminal communication with the bile ducts was identified in five cases on preoperative or intraoperative cholangiographies. Biliary cystic tumors examined in this study were histologically adenoma (one case), adenocarcinoma in situ (six cases), and adenocarcinoma associated with microinvasive mucinous carcinoma (two cases). One case of adenocarcinoma in situ also had the adenoma component (adenocarcinoma in adenoma). Dysplastic mucinous epithelium proliferated in flat, micropapillary and papillary fashions within the intracystic spaces. Intraepithelial neoplasm was observed within non-dilated adjacent bile ducts, suggesting a direct luminal communication between the cystic tumors and the bile duct. Ovarian-like stroma was not observed in their walls in any cases. Immunohistochemically, seven cases expressed MUC1 or MUC2 in the neoplastic biliary epithelium. All cases except one were alive without any evidences of tumor recurrence after total excision (3-156 months after surgery). These clinicopathological features resembled those of intraductal papillary neoplasm of the bile duct, which had been reported as a biliary counterpart of pancreatic intraductal papillary mucinous neoplasm. In conclusion, biliary cystic tumors with bile duct communication could be regarded as intraductal papillary neoplasm with a prominent cystic dilatation of the bile duct and mucin retention, rather than true biliary cystic neoplasms.