Project description:Introduction and importanceIntraductal 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 presentationHereby, 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 discussionThe 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.ConclusionComplete surgical resection (R0) with close postoperative follow-up offers long-term survival.
Project description:Background: Intraductal papillary neoplasm of the bile duct (IPNB) is a rare biliary benign tumor with atypical clinical features and is frequently misdiagnosed. Its treatment is limited and surgical resection is thought to be the only therapeutic option in patients with IPNB. With the aim of increasing the early diagnosis rate of IPNB and providing more therapeutic options for surgeons, we innovatively put forward the concept of combined utilization of SpyGlass and endoscopic endoluminal radiofrequency ablation (ERFA) in the diagnosis and treatment of IPNB. Case Presentation: An 85-year-old woman was referred to our hospital due to right upper quadrant abdominal pain. The image examinations indicated suspicious filling defects at the upper common bile duct. Further evaluation of SpyGlass cholangioscopy showed multiple reddish villous lesions at the left hepatic duct, and SpyBite biopsy under direct visualization demonstrated papillary low-grade dysplasia. In consideration of the advanced age and preference of the patient, the novel ERFA therapy was performed. The procedure was successful without periprocedural complications; the patient recovered uneventfully and was discharged 2 days after the operation. Upon follow-up, the patient was asymptomatic and in good physical condition at 8 months postoperatively. Conclusion: Preliminarily, we demonstrate that the strategy of a combination of SpyGlass and ERAF seems to be a promising, feasible, well-tolerated, and safe management for patients with IPNB. However, more data with larger patient volumes are needed to evaluate its outcomes further.
Project description:IntroductionAn accurate diagnosis of the primary cancer in cases with metastatic lesions is quite important because misdiagnosis may lead to the selection of incorrect adjuvant therapy and worse long-term outcomes after surgery. The metastatic sites associated with the dissemination of colon cancer are well known and normally predictable, which includes the lymphatic, haematogenous, or peritoneal regions, while other locations are quite rare.Presentation of caseIn this report, we present a case of colon cancer with an unusual metastatic pattern mimicking an intraductal papillary neoplasm of the bile duct (IPNB) present in the extra-hepatic bile duct with a cytokeratin (CK)-7-negative and CK-20-positive profile (intestinal type).DiscussionIn the case of this patient who had a history of colon cancer, immunohistochemical staining for the CKs was useful for distinguishing between primary IPNB and colon cancer metastases. We suspect that the metastatic pattern of this case of colon cancer that mimicked IPNB at the extra-hepatic bile duct developed incidentally via the bile stream.ConclusionThis is a rare case of colon cancer metastasis mimicking IPNB at the extra-hepatic bile duct. Our findings also suggest that there may be an incidental 4th metastatic route via the bile stream.
Project description:We encountered a case of wall thickening of the bile duct in a 69-year-old man. endoscopic retrograde cholangiopancreatography (ERCP) was performed for detailed examination. When an area considered to be the healthy bile duct was examined by peroral cholangioscopes (POCS) (SPYGlass DS), the vascular network was observed. POCS-guided, probe-based confocal laser endomicroscopy (pCLE; CholangioFlex, Cellvizio; Mauna Kea Technologies, Paris, France), performed using the fluorescein-dripping method on this area, showed a reticular network of thin dark branching bands, which were presumed to be a collagen bundle or lymphatic vessels according to the Miami Classification. However, 8 ?m-diameter objects thought to be red blood cells were observed inside the bands, which were considered to correspond to the vascular network observed on POCS. A biopsy specimen of this site was taken. The histological examination demonstrated capillaries just beneath the bile duct epithelium. Thus, the histology also suggested the presence of the vascular network. In this study, we obtained findings that cannot be explained in terms of the Miami Classification, which we describe here with a video.
Project description:Video 1Peroral cholangioscopy showed a 20-mm-diameter, stalked, floating, round mass in the hilar region. The surface of the tumor was covered with white fur, and the tumor tissue was partially exposed in a map-like pattern. The mass was penetrable even with a guidewire, and it bled easily. Biopsies were performed using biopsy forceps, and the tumor bled easily even with biopsy.
Project description:Few studies have reported the long-term outcomes of surgical resected intraductal papillary neoplasm of the bile duct (IPNB). Here, we describe the long-term observation and treatment of a case of widespread IPNB.A 57-year-old male was referred to our hospital due to jaundice and dilation of the intrahepatic bile duct. Computed tomography showed dilation and irregularities of the right intrahepatic and extrahepatic bile ducts together with a 3cm nodule in the common hepatic duct. Peroral cholangioscopy revealed mucinous discharge from the ampulla of Vater, which resulted in a diagnosis of IPNB. A biopsy of the nodule and the bile duct revealed papillary adenoma in all of them. Right hepatectomy, caudate lobectomy, extrahepatic bile duct resection, and left hepaticojejunostomy were performed. The nodule was histologically diagnosed as papillary carcinoma in situ, and R0 resection was performed. However, mucus production from the papillary adenoma in the B3 and B4 was observed. We carefully managed the patient's biliary tract by inserting a biliary drainage tube into the segment 2, and he has survived for more than 7 years since the initial treatment.Mucus might be produced after the surgical resection of IPNB even if s surgical margin was benign. Five-year survival rate of benign IPNB was reported from 85% to 100%. That might be caused by difference of the postoperative management of the biliary tract.Careful management of the biliary tract should be performed after surgical resection of IPNB.
Project description:BackgroundActivating point mutations of GNAS at codon 201 have been detected in approximately two thirds of intraductal papillary mucinous neoplasms (IPMNs) of the pancreas. Intraductal papillary neoplasms of the bile ducts (IPNBs) morphologically resemble pancreatic IPMNs. This study sought to assess the mutational status of GNAS at codon 201 in IPNBs.MethodsThirty-four patients were included. DNA from microdissected IPNBs was subjected to a polymerase chain reaction and ligation method for the detection of GNAS mutations at codon 201 and of KRAS mutations at codon 12. Mutational status was compared with clinical and pathologic data.ResultsThe IPNBs had a median diameter of 3.5 cm and were located intrahepatically (n= 6), extrahepatically (n= 13), both intra- and extrahepatically (n= 4) or in the gallbladder (intracystic papillary neoplasms, n= 11). Most exhibited pancreatobiliary differentiation (n= 20), high-grade dysplasia (n= 26) and an associated adenocarcinoma (n= 20). Analysis of GNAS codon 201 identified only one mutant sample in a multifocal intestinal subtype intrahepatic IPNB with high-grade dysplasia. Six lesions harboured a KRAS codon 12 mutation.ConclusionsGNAS codon 201 mutations are uncommon in IPNBs, by contrast with pancreatic IPMNs. More comprehensive molecular profiling is needed to uncover the pathways involved in IPNB development.
Project description:Based on consensus guidelines, surgical resection of branch duct intraductal papillary mucinous neoplasm (BD-IPMN) is indicated in patients with symptoms of cyst size >or=30 mm, intramural nodules, or dilated main pancreatic duct greater than 6 mm. The aim of this study was to determine the cost effectiveness of consensus guideline implementation in the management of BD-IPMN.We developed a decision analytic model to compare the costs and effectiveness of three management strategies for a cohort of 60-year-old patients with branch duct IPMN: (1) surveillance using consensus guidelines for surgical resection (surveillance strategy), (2) surgical resection based on symptoms without surveillance (no surveillance strategy), and (3) immediate surgery (surgery strategy). The primary outcomes were quality-adjusted life years (QALYs), cost, and incremental cost-effectiveness ratio (ICER). Sensitivity analysis was performed over a wide ranges of estimates.The no surveillance strategy was the least costly, but also the least effective, while the surgery strategy was the most costly and most effective. Compared to the no surveillance strategy, the surveillance strategy cost an additional $20,096 per QALY. The incremental cost-effectiveness ratio of the surgery strategy compared with the surveillance strategy was $132,436 per QALY. In a probabilistic sensitivity analysis, if society was willing to pay $50,000 per quality-adjusted life year gained, then 88.1% of patients using the surveillance strategy would be within budget.Immediate surgery is the most effective, but may be prohibitively expensive. The surveillance strategy is a cost-effective option compared to no surveillance.