An Icteric Type Hepatocellular Carcinoma with No Detectable Tumor in the Liver: Report of a Case

A 70-year-old man was admitted to our hospital with obstructive jaundice. Computed tomography revealed a tumor in the left intrahepatic bile duct extending to the common bile duct without any significant lesions in the liver. Cholangiography showed a filling defect due to an intraductal tumor. Cytology of the bile juice was negative and tumor markers were carcinoembryonic antigen 5.7 ng/ml, carbohydrate antigen 19-9 49 U/ml, α-fetoprotein 9 ng/dl, and PIVKA-II 19 200 AU/ml. With a preoperative diagnosis of hilar bile duct carcinoma, a laparotomy was performed. The common bile duct was filled with a tumor and it extended into the bilateral intrahepatic bile ducts. The intraductal tumor was removed together with the extrahepatic bile ducts. An intraoperative histological examination of the tumor showed a well-differentiated hepatocellular carcinoma. No lesions were detected in the liver by ultrasonography, palpation during the operation, or a computed tomography scan after the operation. At 1 year postoperatively, no recurrence has been seen in this patient.     

Spindle Cell-Type Undifferentiated Carcinoma of the Common Bile Duct of the Hepatic Hilus: Report of a Case

Spindle cell-type undifferentiated carcinoma arising from the extrahepatic bile duct is extremely rare. We herein report a case of this type of carcinoma in the common bile duct of the hepatic hilus. A 59-year-old man was admitted to our hospital complaining of jaundice. The laboratory data revealed an elevation of the serum carbohydrate antigen 19–9 level. Cholangiography revealed a complete obliteration of the left hepatic bile duct and stenosis of the bile duct from the superior to the right hepatic bile duct. Computed tomography showed the tumor to measure 15 × 12 mm in the hepatic hilus, with the obliteration of the right to main trunk of the portal vein and a swollen lymph node in the hepato-duodenum ligament. Arteriography revealed a kink of the right hepatic artery; therefore an encasement of the right hepatic artery was suspected. We preoperatively diagnosed hilus bile duct carcinoma and scheduled a right trisection hepatectomy. Intraoperative frozen sections taken from the tumor and tissues around the hepatic arteries showed spindle and inflammatory cells; therefore an inflammatory pseudotumor was diagnosed intraoperatively. As the right hepatic bile duct was occluded, a right lobe hepatectomy was performed. However, a permanent section revealed both spindle cells and poorly differentiated tubular adenocarcinoma cells positive for CAM5.2, AE1/AE3, and vimentin. On the basis of these findings, the tumor was finally diagnosed to be spindle cell-type undifferentiated carcinoma. Unfortunately, the patient died of pulmonary infarction 11 days after the operation.     

An unusual case of jaundice: Biliary tumor thrombus in fibrolamellar hepatocellular carcinoma

BackgroundFibrolamellar hepatocellular carcinoma (FL-HCC) is a rare and unique variant of hepatocellular carcinoma (HCC) whose presentation remains inadequately described. We present a resectable case of FL-HCC which involved tumor thrombus of the common bile duct.PresentationA 27 year-old male presenting with jaundice, abdominal pain, vomiting, hepatic dysfunction and hyperbilirubinemia was found to have a large liver mass and lymphadenopathy on preoperative imaging. A right hepatectomy with perihepatic lymph node dissection and cholecystectomy was performed. Intraoperative cholangiogram demonstrated common bile duct (CBD) obstruction. CBD exploration revealed biliary tumor thrombus relieved with biliary thrombectomy.DiscussionFL-HCC can initially present with invading obstructing biliary tumor thrombus of the CBD causing jaundice.ConclusionPreoperative surgical approach should consider CBD exploration on an individual basis for underlying obstructive biliary tumor thrombus.     

Small pancreatic carcinoma misdiagnosed as superficially spreading cholangiocarcinoma

We report a case of small pancreatic carcinoma misdiagnosed as superficially spreading cholangiocarcinoma using percutaneous transhepatic cholangioscopy (PTCS). The patient was a 72-year-old man admitted to a local hospital with obstructive jaundice. The patient underwent percutaneous transhepatic biliary drainage and PTCS. He was referred to our hospital with a diagnosis of superficially spreading cholangiocarcinoma. Cholangiography revealed a stenosis of the common bile duct, and also revealed some irregularities from the common hepatic duct to the left hepatic duct, suggesting a superficial spread of cancer. No pancreatic tumor was identified by endoscopic retrograde pancreatography or by enhanced computed tomography. Cholangioscopy disclosed an elevated tumor with torsional vessels and granular mucosal lesions, which were extended to the left hepatic duct. Repeated cholangioscopic biopsies of the bile duct mucosa revealed adenocarcinoma. The patient was diagnosed with superficially spreading cholangiocarcinoma extending to the left hepatic duct and the right anterior hepatic duct. Left trisectionectomy combined with pancreatoduodenectomy was performed. The cut surface of the resected specimen showed a pancreatic head tumor that was 8 mm in diameter. Histological findings of the resected specimen revealed adenocarcinoma arising from the pancreatic head with invasion in the common bile duct. Additionally, extensive inflammatory granulation tissue was observed along the surface of the bile duct, without any evidence of carcinoma. This case implies to us that the results of PTCS, even after repeated biopsies, should be interpreted with great caution.     

A resected case of a small hepatocellular carcinoma developing within the bile duct

We experienced a resected case of a small hepatocellular carcinoma, which required differential diagnosis from intrahepatic cholangiocellular carcinoma. The patient was a 76-year-old man. While his course had been being observed because of hepatitis C antibody-positive liver cirrhosis, ultrasonographic examination of the abdomen revealed dilation of biliary branches in the anterior segment of the liver and a hyperechoic mass 10mm in diameter at the origin of the branch. A dynamic computed tomography scan showed a high-density tumor in the early phase. After embolization of the right branch of the portal vein, resection of the right lobe of the liver and the extrahepatic bile duct was performed. A resected specimen showed a white-colored mass 8mm in diameter at the origin of the anterior segmental biliary branch. In the pathological findings, the diagnosis was a poorly differentiated hepatocellular carcinoma with strong nuclear atypia; the tumor filled the bile duct, forming a trabecular structure. The immunohistological stains of the tumor were positive for cytokeratin (CK) 8, CK18, and HepParl and negative for alpha-fetoprotein, carcinoembryonic antigen, CA19-9, CK7, CK19, and CK20. There was atypia in the biliary lining epithelium adjacent to the tumor, and the hepatocellular carcinoma may have developed from the biliary epithelium.     

Clinical and pathological features of primary carcinoma of the cystic duct

Background  According to Farrar’s criteria, a tumor restricted to the cystic duct is defined as cystic duct carcinoma, but this definition excludes advanced carcinoma originating from the cystic duct. Patients and methods  For the purpose of this study, primary cystic duct carcinoma was defined as a tumor originating from the cystic duct. We investigated the clinicopathological features of 15 cystic duct carcinomas, including 13 that did not fit Farrar’s criteria, and compared them with those of 52 cases of gallbladder carcinoma and 161 cases of extrahepatic bile duct carcinoma. Results  The incidence of primary cystic duct carcinoma was 6.6% among all malignant biliary tumors. The main symptom was jaundice in 67% of cases. The operative procedures employed ranged from cholecystectomy to hepatopancreatoduodenectomy. The cases of cystic duct carcinoma and bile duct carcinoma showed a high frequency of perineural infiltration. The overall 5-year survival rate of the 15 patients was 40%. Conclusion  Patients with advanced cystic duct carcinoma show a high frequency of jaundice and perineural infiltration. Our data suggest that cystic duct carcinoma may be considered a distinct subgroup of gallbladder carcinoma. Radical surgery is necessary for potentially curative resection in patients with advanced cystic duct carcinoma.     

Villous tumor of the papilla of Vater presenting with hypopotassemia

We report a case of villous tumor of the papilla of Vater associated with hypopotassemia. The patient was a 73-year-old woman who presented with jaundice and fever. She had a history of diabetes mellitus and liver dysfunction. Laboratory studies revealed that levels of total bilirubin, alkaline phosphatase, and C-reactive protein, and the white blood cell count were elevated (suggestive of cholangitis) and that the serum potassium level was markedly reduced, to 1.9 mEq/l (normal value 3.5–5.0 mEq/l). Duodenoscopy showed a villous tumor arising in the papilla of Vater. Percutaneous transhepatic biliary drainage was performed. Approximately 700–1500 ml of bile with viscous mucoid fluid was drained daily. Percutaneous transhepatic cholangioscopy showed a papillary lesion in the distal common bile duct. Biopsied specimens from both percutaneous transhepatic cholangioscopy and duodenoscopy disclosed tubulovillous adenoma. Endoscopic ultrasonography showed that the tumor had spread to the main pancreatic duct as well as to the common bile duct. The patient underwent pylorus-preserving pancreaticoduodenectomy. Pathology examination disclosed well differentiated adenocarcinoma, carcinoma in situ, in tubulovillous adenoma. The cancer cells were observed at the bottom of the tumor spreading in the common bile duct. This is a rare case of a patient presenting with hypopotassemia associated with a tubulovillous tumor of the papilla of Vater that secreted mucoid material.     

胆囊癌引起梗阻性黄疸的原因分析

目的 探讨胆囊癌引起梗阻性黄疸的病因.方法 回顾分析我院2000 ～ 2009年10年间84例胆囊癌患者资料.结果 42例出现黄疸,其中肿瘤侵犯肝脏或发生肝内转移9例,肿瘤侵犯肝总管、胆总管11例,肝十二指肠韧带、胰头部肿大淋巴结7例,胆总管结石4例,两种及两种以上上述情况的11例.结论 胆囊癌引起梗阻性黄疸的原因多样...     

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目的 探讨不同类型原发性肝癌伴梗阻性黄疸的原因及其治疗方法。方法 分析原发性肝癌伴梗阻性黄疸的病人15例。其中肝癌引起胆管癌栓7例。肝癌压迫或侵犯肝门部胆管8例。其中手术10例，清除肝癌原发灶，解除胆管梗阻，非手术5例，先行减轻黄疸处理，然后行TAE，PEI等综合治疗。结果 手术切除病灶，延长生存期，而非手术处理组也明显改善症状，疗效显著，随访1-24个月（平均9个月）3例死亡，12例仍存活。结论 临床上有必要对原发性肝癌伴梗阻性黄疸进行积极治疗，以改善生活质量，延长生存期。     

Curative resection of hepatocellular carcinoma with intrabile duct tumor growth mimicking hilar bile duct carcinoma

A 62-year-old Japanese male was admitted with obstructive jaundice and underwent percutaneous transhepatic cholangiodrainage (PTCD). An initial diagnosis was made of hilar bile duct carcinoma, based on demonstrated irregular stenosis of the hilar hepatic bile ducts without obvious tumor within the liver and negative alpha-fetoprotein (AFP). Enhanced computed tomography (CT) showed an irregular low density area around the hepatic hilum and the umbilical portion of the portal vein, suggesting carcinomatous invasion with blood flow disturbances. In contrast, intraoperative ultrasonography (US) raised the suspicion of an ill-defined lesion in Couinaud's segment 2 (segment 2) and intrabile duct tumor formation. A radical extended left and caudate lobectomy of the liver was successfully performed, with additional resection of extrahepatic bile duct and enbloc resection of regional lymph nodes. Unexpectedly, histological analysis of the resected specimen showed the final diagnosis to be hepatocellular carcinoma (HCC) of segment 2 with intrabile duct tumor growth. This case demonstrates that HCC with intrabile duct tumor growth toward the heptic hilum can mimic hilar bile duct carcinoma, when the tumor itself is equivocal on preoperative imaging and AFP is negative. In such cases, intraoperative US and guided biopsy may be of value for definitive diagnosis and selection of the optimal procedure.     

Malignant Carcinoid Tumor of the Common Bile Duct: Report of a Case

Carcinoid tumors of the extrahepatic bile duct are exceedingly rare and account for between 0.1% and 2% of all gastrointestinal carcinoid tumors, with most reported cases arising from the gallbladder. We herein present what we believe is only the 47th reported case of a primary carcinoid tumor occurring in the extrahepatic bile ducts. A 67-year-old woman sought treatment for obstructive jaundice accompanied by epigastric pain. Laboratory and imaging studies gave results that were consistent with a malignant obstruction in the common bile duct. We performed a pyrolus-preserving pancreaticoduodenectomy. Pathologically, an ill-demarcated mass was noted in the common bile duct measuring 1.6 × 1.5 × 0.5 cm in size. The tumor had invaded the adjacent pancreatic tissues. Immunohistochemically, the mass demonstrated chromogranin, synaptophysin, and CD56 positivity. The final pathologic diagnosis was well-differentiated carcinoid tumor of a malignant nature. The patient, who underwent a curative surgical resection, was alive and disease-free at the time of writing.     

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??Diagnosis and management of hepatocellular carcinoma with biliary tumor thrombi YIN Xiao-yu. Department of Pancreato-Biliary Surgery??the First Affiliated Hospital??Sun Yat-Sen University??Guangzhou 510080, China
Abstract Hepatocellular carcinoma (HCC) with biliary tumor thrombi is not common. Biliary tumor thrombi usually arise from the intrahepatic bile duct which is invaded by HCC, and gradually extend towards the hilar bile ducts, and even common bile duct. It eventually leads to obstructive jaundice, and hemobilia occasionally. HCC with biliary tumor thrombi sometimes can be misdiagnosed as cholangiocarcinoma. Correct diagnosis is important. Aggressive surgical resection is helpful to prolong the patient’s survival time??and improve the long-term outcome.     

Primary extranodal non-Hodgkin’s lymphoma of the common bile duct manifesting as obstructive jaundice: Report of a case

Primary non-Hodgkin’s lymphoma (NHL) of the common bile duct (CBD) manifesting as obstructive jaundice is extremely rare: to our knowledge, only 22 cases of primary NHL arising from the CBD have been reported. The patient in this case report was a 63-year-old man who presented with obstructive jaundice. Abdominal sonography, positron emission tomography, and computed tomography showed a mass with abnormal 18-fluorodeoxyglucose uptake in pancreatic head. Magnetic resonance cholangiopancreatography demonstrated a strictured segment of the CBD with proximal bile duct dilatation. We performed pancreaticoduodenectomy for a presumptive diagnosis of pancreatic head carcinoma or cholangiocarcinoma of the CBD. However, the histological diagnosis was a primary, diffuse, large B-cell lymphoma of the CBD. He received three courses of combination chemotherapy, including rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP). The patient remains well, without evidence of tumor recurrence, 8 months after surgery. In summary, primary NHL of the CBD, despite its rarity, should be considered in the differential diagnosis of obstructive jaundice. An accurate histopathologic diagnosis and complete surgical resection, followed by combination chemotherapy plus rituximab may be effective.     

原发性肝癌伴胆管癌栓致阻塞性黄疸的外科治疗

目的探讨原发性肝癌伴胆管癌栓致阻塞性黄疸的外科手术治疗及其治疗效果。方法回顾性对15例原发性肝癌伴胆管癌栓致阻塞性黄疸行外科手术治疗的总结和分析。结果行左半肝切除 胆总管切开取癌栓术5例,行肿瘤切除 胆总管切开取癌栓术7例,行胆总管切开取癌栓 肝总动脉结扎3例,术后随访2年,平均生存时间为14.5个月,最长存活23个月。结论外科治疗明显提高了患者生活质量,延长了生存时间。     

无黄疸期肝外胆管癌的诊治体会

目的 了解未出现黄疸的肝外胆管癌在诊治方面与有黄疸者有无差别。方法 回顾性地总结了湖南医科大学附属湘雅医院外科1984年无月至1998年12月经手术治疗的86例肝外胆管癌。按术前血清总胆红素水平分为黄疸组与无黄疸组。分别了解其临床表现,生化检查、影像学检查、肿块部位、病理分型及手术切除情况。结果 在没有明显手术禁证的肝外胆管癌中无黄疸期刖外胆管癌占本组总数的16.2%;在无黄疸组各种生化指标（如HB,ALB,AKP,γ-GT等）及B超检查已有与黄疸组类似的改变,而且两组肝外胆管癌的肿块部位,病理类型及手术切除率亦无显著差异;无黄疸期肝外胆管癌结石合并率较黄疸期者明显为高。结论 不能单纯依靠有无黄疸而对肝外胆管癌的进展程度及预后进行评价和预测应提高对此疾病的警惕,以便及早发现病变。     

Small cell carcinoma of the cystic duct: A case report

Small cell carcinoma usually involves the lung and rarely affects the biliary tract, especially the cystic duct. In this article we report a case of small cell carcinoma of the cystic duct in a 46-year-old Japanese man. The patient presented with abdominal pain and jaundice. Imaging showed a small nodule in the cystic duct invading the common bile duct with dilatation of the proximal biliary tree. The hepatic artery and portal vein were free from invasion. Extended right hepatic lobectomy, cholecystectomy, and resection of the extrahepatic proximal bile ducts were performed together with lymph node dissection under the tentative diagnosis of carcinoma of the cystic duct. Histopathologic examination of the resected specimen revealed small cell carcinoma arising in the cystic duct and extending into the common bile duct. The postoperative clinical course was uneventful, and the patient is doing well without any signs of recurrence 1 year after the operation. To our knowledge this is the first documented case of a small cell carcinoma arising in the cystic duct.     

肝细胞癌合并胆管癌栓的诊断与治疗

肝细胞癌（简称肝癌）合并胆管癌栓不常见。胆管癌栓通常是由肝癌侵入其所在部位的肝内胆管而形成并沿肝内胆管向肝门部胆管甚至胆总管生长,最终导致梗阻性黄疸或合并胆道出血。肝癌合并胆管癌栓并不是一种终末期疾病,有时会被误诊为胆管癌,及时正确诊断至关重要。积极手术切除治疗有助于延长生存时间、改善远期预后。     

Surgical Treatment for Biliary Carcinoma Arising After Pancreatoduodenectomy

The clinicopathological features and surgical treatment of biliary carcinoma around the major hepatic duct confluence arising after pancreatoduodenectomy (PD) due to initial bile duct carcinoma are described in three patients. Occurrence of biliary carcinoma more than 12 years after initial surgery and a histological finding of cholangiocellular carcinoma mixed with hepatocellular carcinoma suggested metachronous incidence of biliary carcinoma after PD. Extended right hemihepatectomy with complete removal of the residual extrahepatic bile duct and segmental, resection of the jejunal loop were carried out safely without operative death or severe postoperative complications. Two patients died of tumor recurrence 6 months after surgery, and the remaining patient is currently living a normal life without evidence of recurrence 17 months after surgery. These surgical procedures are a therapeutic option in patients with biliary carcinoma around the major hepatic duct confluence arising after PD.     

Neuroendocrine tumor of the extrahepatic bile duct: A case report

IntroductionNeuroendocrine tumors (NETs) of the extrahepatic bile ducts are extremely rare neoplasms arising from endocrine cells and have variable malignant potential. They most commonly occur in young females and usually present with painless jaundice.Presentation of caseHere we present the case of an asymptomatic 57-year-old woman with NET of the common bile duct that was incidentally discovered on abdominal ultrasound during a medical examination. She was admitted to our hospital with a diagnosis of hepatic hilar tumor. Computed tomography revealed the tumor surrounding the hepatic hilum and duodenum. Magnetic resonance cholangiopancreatography revealed a filling defect of the common bile duct with morphology suggestive of external compression. Endoscopic ultrasound confirmed a submucosal tumor of the duodenal bulb measuring 30 × 20 mm in size. The patient qualified for surgery with a preoperative diagnosis of submucosal tumor of the duodenal bulb. Intraoperative examination revealed that the tumor location involved the common bile duct and/or cystic duct with no signs of invasion to other organs or metastatic lymph nodes. Excision of the biliary ducts and tumor was followed by Roux-en-Y anastomosis. Histological results showed NET grade 1.DiscussionPreoperative diagnosis of NETs is difficult because of their rarity. A definitive diagnosis is usually established intraoperatively or after histopathological evaluation.ConclusionFor these tumors, surgical resection is currently the only treatment modality for achieving a potentially curative effect and prolonged disease-free survival.     

肝癌合并胆道癌栓(附10例报告)

目的 探讨肝癌合并胆道癌栓的发生率、发生机制及治疗问题。方法 回顾性分析10例肝癌合并胆道癌栓病例的临床特点、手术方法及治疗效果。结果 病人行手术治疗组存活时间分别为18月、20月、23月,明显高于非手术治疗组。结论 本类型肝癌采取积极主动的综合手术方式进行处理,预后佳。