Prognosis of hepatocellular carcinoma with bile duct tumor thrombus after R0 resection:a matched study

BACKGROUND： Hepatocellular carcinoma （HCC） with bile duct tumor thrombus （BDTT） is rare. The present study aimed to determine post-surgical prognoses in HCC patients with BDTT, as outcomes are currently unclear. METHODS： We compared the prognoses of 110 HCC patients without BDTT （group A） to 22 cases with BDTT （group B）. The two groups were matched in age, gender, tumor etiology, size, number, portal vascular invasion, and TNM stage. Additionally, 28 HCC patients with BDTT were analyzed to identify prognostic risk factors. RESULTS： The 1-, 3-, and 5-year overall survival rates were 90.9%, 66.9%, and 55.9% for group A and 81.8%, 50.0%, and 37.5% for group B, respectively. The median survival time in groups A and B was 68.8 and 31.4 months, respectively （P=0.043）. The patients for group B showed higher levels of serum total bilirubin, alanine aminotransferase and gammaglutamyl transferase, a larger hepatectomy range, and a higher rate of anatomical resection. In subgroup analyses of patients with BDTT who underwent R0 resection, TNM stage Ⅲ-Ⅳ was an independent risk factor for overall survival; these patients had worse prognoses than those with TNM stage Ⅰ-Ⅱ after R0 resection （hazard ratio=6.056, P=0.014）. Besides, univariate and multivariate analyses revealed that non-R0 resection and TNM stage Ⅲ-Ⅳ were independent risk factors for both disease-free survival and overall survival of 28 HCC patients with BDTT. The median overall survival time of patients with BDTT who underwent R0 resection was longer than that of patients who did not undergo R0 resection （31.0 vs 4.0 months, P=0.007）.CONCLUSIONS： R0 resection prolonged survival time in HCC patients with BDTT, although prognosis remains poor. For such patients, R0 resection is an important treatment that determines long-term survival.

Surgical treatment of hepatocellular carcinoma with bile duct tumor thrombi

AIM: To study the surgical treatment effect and outcome of hepatocellular carcinoma (HCC) with bile duct tumor thrombi (BDTT). METHODS: Fifty-three consecutive HCC patients with BDTT admitted in our department from July 1984 to December 2002 were reviewed retrospectively. The clinical data, diagnostic methods, surgical procedures and outcome of these patients were collected and analyzed. RESULTS: One patient rejected surgical treatment, 6 cases underwent percutaneous transhepatic cholangial drainage (PTCD) for unresectable primary disease, and the other 46 cases underwent surgical operation. The postoperative mortality was 17.6%, and the morbidity was 32.6%. Serum total bilirubin levels of these patients with obstructive jaundice decreased gradually after surgery. The survival time of six cases who underwent PTCD ranged from 2 to 7 mo (median survival of 3.7 mo). The survival time of the patients who received surgery was as follows: 2 mo for one patient who underwent laparotomy, 5-46 mo (median survival of 23.5 mo, which was the longest survival in comparison with patients who underwent other procedures, P=0.0024) for 17 cases who underwent hepatectomy, 5-17 mo (median survival of 10.0 mo) for 5 cases who underwent HACE, 3-9 mo (median survival of 6.1 mo) for 11 cases who underwent simple thrombectomy and biliary drainage, and 3-8 mo (median survival of 4.3 mo) for four cases who underwent simple biliary drainage. CONCLUSION: Jaundice caused by BDTT in HCC patients is not a contraindication for surgery. Only curative resection can result in long-term survival. Early diagnosis and surgical treatment are the key points to prolong the survival of patients.

Markers of bile duct tumors

Biliary tract carcinomas are relatively rare,representing less than 1%of cancers.However,their incidence has increased in Japan and in industrialized countries like the USA.Biliary tract tumors have a poor prognosis and a high mortality rate because they are usually detected late in the course of the disease;therapeutic treatment options are often limited and of minimal utility.Recent studies have shown the importance of serum and molecularmarkers in the diagnosis and follow up of biliary tract tumors.This review aims to introduce the main features of the most important serum and molecular markers of biliary tree tumors.Some considerable tumor markers are cancer antigen 125,carbohydrate antigen 19-9,carcinoembryonic antigen,chromogranin A,mucin 1,mucin 5,alpha-fetoprotein,claudins and cytokeratins.

Carcinosarcoma of common bile duct:A case report

BACKGROUND Carcinosarcomas of the common bile duct(CBD)are an extremely rare finding in the clinical setting.Based on a review of 12 literatures,3 cases had the imaging features of ossification.Carcinosarcomas are prone to distant metastasis,as they possess clinical features of both carcinoma and sarcoma,and generally have with a poor prognosis.Due to the small number of cases reported,clinical experience in the diagnosis and treatment of the disease is lacking.CASE SUMMARY The patient was a 75-year-old woman who had experienced recurrent chills with nausea and vomiting for 3 mo.Computed tomography,magnetic resonance imaging,endoscopic ultrasonography and endoscopic retrograde cholangiopancreatography led to the diagnosis of malignant tumor of the CBD.The patient ultimately underwent cholecystectomy,CBD resection,and choledochojejunostomy.Postoperative pathological examination revealed carcinosarcoma of the CBD,and the latest follow-up showed that the patient is recovering well.Based on previous case reports,some carcinosarcoma has ossification characteristics in imaging.If it is misdiagnosed as biliary calculi,the use of laser lithotripsy in surgery may lead to tumor diffusion.Choledochoscopy and narrow band staining of mucosa are very important for diagnosis.CONCLUSION We herein present a rare case of carcinosarcomas of the CBD,we found the tumours may have imaging features of polypoid growth and ossification only when the sarcomal components are bone differentiation,while show soft tissue shadow when non bone differentiation.Confirmation of diagnosis depends greatly upon postoperative pathological examination and the adjuvant treatment has not been established,which leads to the poor prognosis.

SpyGlass in Diagnosis of Hepatocellular Carcinoma with Right Hepatic Duct Tumor Thrombus Hemorrhage: A Case Report

Hepatocelluar carcinoma presenting as a biliary duct tumor thrombus is a relatively rare entity, with poor prognosis. The primary clinical manifestation of this disease is obstructive jaundice, which can often be misdiagnosed. A 59-year-old female patient was admitted with sudden onset of abdominal pain. Laboratory tests suggested obstructive jaundice, and enhanced magnetic resonance imaging of the upper abdomen did not show obvious biliary dilatation. Endoscopic ultrasound and endoscopic retrograde cholangiopancreatography suggested an occupying lesion in the upper bile duct. SpyGlass and biopsy finally confirmed hepatocellular carcinoma with right hepatic duct tumor thrombus hemorrhage. The SpyGlass Direct Visualization System, as an advanced biliary cholangioscopy device, showed the advantages of single-person operation as well as easy access to and visualization of the lesion.

The benefit of curative liver resection with a selective bile duct preserving approach for hepatocellular carcinoma with macroscopic bile duct tumor thrombus

Background:Hepatocellular carcinoma(HCC)presenting with macroscopic bile duct tumor thrombus(BDTT)is an uncommon event.The role of a curative hepatic resection and associated long-term outcomes remain controversial.In addition the necessity for bile duct resection is still unclear.The aim of this study was to evaluate outcomes of hepatectomy with a selective bile duct preservation approach for HCC with BDTT in comparison to outcomes without BDTT.Methods:A total of 22 HCC with BDTT patients who had undergone curative hepatic resection with a selective bile duct preservation approach at our institute were retrospectively reviewed.These were compared to group of 145 HCC without BDTT patients.The impact of curative surgical resection and BDTT on clinical outcomes and survival after surgical resection were analyzed.Results:All HCC with BDTT cases underwent major hepatectomy vs.32.4%in the comparative group.Bile duct preservation rate was 56.5%.The 1-,3-and 5-year survival rates of HCC with BDTT patients in comparison to the HCC without BDTT group were 81.8%,52.8%and 52.8%vs.73.6%,55.6%and 40.7%(P=0.804)respectively.Positive resection margin,tumor size≥5 cm and AFP≥200 IU/mL were significant risk factors regarding overall survival.However,it is unclear whether presence of a bile duct tumor thrombus has an adverse impact on either recurrence free survival or overall survival.Conclusions:Bile duct obstruction from tumor thrombus did not necessarily indicate an advanced form of disease.Tumor size and AFP had greater impact on long-term outcomes than bile duct tumor thrombus.Major liver resection with a selective bile duct preserving approach in HCC with BDTT can achieve favorable outcomes comparable to those of HCC without BDTT in selected patients.

Uptake of bacterial lipopolysaccharide and expression of tumor necrosis factor α mRNA in isolated rat intrahepatic bile duct epithelial cells *

AIM To study the uptake of bacterial lipopolysaccharides (LPS) and expression of tumor necrosis factor α mRNA (TNF α mRNA) with cultured rat intrahepatic bile duct epithelial cells.

Carcinoid tumor of the common bile duct: A rare complication of von Hippel-Lindau syndrome

Von HippeI-Lindau syndrome （VHL） is a rare autosomal-dominant, inherited familial cancer syndrome. Hemangioblastomas, pheochromocytomas and renal carcinoma are the frequent reported VHL tumors. Neuroendocrine tumors have also been described, mostly in the pancreas and rarely in the biliary trees. We report the second case of bile duct carcinoid in a 31-year-old VHL woman. She underwent right adrenalectomy for a pheochromocytoma in the past. She also had a positive family history of phenotypic expression of VHL syndrome. The patient presented with biliary colic. Endoscopic retrograde cholangio-pancreatography showed intraluminal bile duct mass. Surgical exploration identified a beige nodular lesion that was a carcinoid tumor on histology. This new association should be clarified by further genetic investigations.

Tumors with macroscopic bile duct thrombi in non-HCC patients:Dynamic multi-phase MSCT findings

Non-hepatocellular carcinoma （non-HCC） with macroscopic bile duct tumor thrombus （BDTI） formation is rare, few radiological studies have been reported. In this case report, we retrospectively analyzed the imaging findings of three cases of non-HCC with macroscopic BDTT on dynamic enhanced multislice computed tomography （MSCT） scan. One case of primary hepatic carcinosarcoma was presented as a solitary, large welldefined tumor with significant necrotic changes. One case of liver metastasis from colon cancer was presented as a Iobulated, large ill-defined tumor. One case of intraductal oncocytic papillary neoplasm involved the entire pancreas, presented as a cystic and solid mass with multilocular changes （the individual Ioculi were less than 5.0 mm in diameter）. The bile duct was dilated due to expansible growth of the BDFE in all three patients. The BDTT was contiguous with hepatic or pancreatictumor, and both of them showed the same enhancement patterns on dynamic contrast-enhanced computed tomography scan： early enhancement in the hepatic arterial phase and a quick wash out of contrast agent in the portal and equilibrium phases. Macroscopic BDTT- in non-HCC patient is rare, dynamic enhanced MSCT scan may be valuable in the diagnosis of non-HCC with BDTT.

Intraductal papillary bile duct adenocarcinoma and gastrointestinal stromal tumor in a case of neurofibromatosis type 1

We report our experience with a synchronous case of gastrointestinal stromal tumor(GIST) and intraductal papillary neoplasm of the bile duct(IPNB) in anelderly woman with neurofibromatosis type 1(NF-1). A 72-year-old woman presented with a 2-mo history of right upper abdominal pain unrelated to diet and indigestion. Fourteen years earlier, she had been diagnosed with NF-1, which manifested as café au lait spots and multiple nodules on the skin. Computed tomography(CT) revealed a multilocular low-density mass with septation, and mural nodules in the right hepatic lobe, as well as a 1.7-cm-sized well-demarcated enhancing mass in the third portion of the duodenum. The patient subsequently underwent right hepatectomy and duodenal wedge resection. We present here the first report of a case involving a synchronous IPNB and GIST in a patient with NF-1. Our findings demonstrate the possibility of various tumors in NF-1 patients and the importance of diagnosis at an early

Benign giant-cell tumor of the common bile duct:A case report

Primary giant-cell tumors rarely arise in the common bile duct. We herein report a case of primary giant-cell tumor of the common bile duct. The patient was an 81-year-old male who was diagnosed with a well-defined 1.2-cm mass projecting into the lumen of the middle common bile duct. Excision of the gallbladder and extrahepatic bile duct and a Roux-en-Y cholangiojejunostomy were performed. Histologically, the tumor had no association with carcinomas of epithelial origin and was similar to giant-cell tumors of the bone. The tumor consisted of a mixture of mononuclear and multinucleated osteoclast-like giant cells. The mononuclear cells showed no atypical features, and their nuclei were similar to those of the multinucleated giant cells. CD68 was expressed on the mononuclear and multinucleated osteoclast-like giant cells, whereas CD163 immunoreactivity was restricted to the mononuclear cells. Six months after the operation, the patient was still alive and had no recurrence. The interest of this case lies in the rarity of this entity, the difficulty of preoperative diagnosis, and this tumor’s possible confusion with other malignant tumors.

Pediatric intrahepatic bile duct adenoma-rare liver tumor: A case report

BACKGROUND Intrahepatic bile duct adenoma(BDA)is one of the rarest of the rare benign tumors of the liver in the pediatric age group.It arises from the epithelial lining of intrahepatic bile ducts.The overall incidence of BDA is 1.3%of all primary benign liver tumors.Few case reports of this rare tumor occurring in adult population are present in the literature and to date,only one pediatric case has been reported worldwide.CASE SUMMARY An 18-month-old male child presented with chief complaints of mass per abdomen for 8 mo.Computerized tomography abdomen revealed a well-defined exophytic solid tumor arising from the posteroinferior margin of the right lobe of the liver with heterogenous enhancement and cystic changes,suggesting a suspicion of hepatoblastoma.Non-anatomical liver resection was done and a tumor of 10 cm×9.5 cm was excised.Histopathology of the specimen was conclusive with the diagnosis of intrahepatic bile duct adenoma,which was further supported by immunohistochemistry panel testing.The post-operative period was uneventful.On follow-up,the child was doing well and had no postoperative complication.CONCLUSION Intrahepatic bile duct adenoma in children is very rare and to date only one case has been reported.This is the second pediatric case of intrahepatic bile duct adenoma in the world.Additionally this is the first ever case of such a large tumor presenting in a child.

Non-functioning well-differentiated neuroendocrine tumor of the extrahepatic bile duct:an unusual suspect?

BACKGROUND:Neuroendocrine tumors(NETs)arising in the biliary tree are extremely rare,and 37 cases were identified in the English literature. METHODS:A well-differentiated NET was found arising from the junction of the cystic and common hepatic ducts, in a 51-year-old male presenting with pedal edema and weight loss with abnormal liver enzymes and a normal serum bilirubin level.No mass was seen on radiological imaging and biopsy of the liver was suggestive of an early cholangiopathy.A bile leak complicating the liver biopsy led to an ERCP that demonstrated a filling defect suggestive of a mass in the common bile duct(CBD). RESULTS:He underwent a successful excision of the tumor with a Roux-en-Y hepaticojejunostomy.The diagnosis of NET was made on histological and immunohistochemical analysis of the resected specimen.He remains well and disease free 22 months after surgery. CONCLUSIONS:Recognition of biliary NET continues to be a challenge and an increased awareness of these tumors in rare sites will result in optimal management of these tumors.

Neuroendocrine Tumor Arising from Common Bile Duct: A Case Report and Literature Review

Neuroendocrine tumor (NET) arising from common bile duct (CBD) is a rare disease entity, whose origin is currently in discussion with various theories. We present a case of well-defined solid, exophytic and arterial hypervascular mass arising from CBD with relatively mild dilated central intrahepatic bile ducts in 50-year-old man, which was surgically confirmed to be extrahepatic biliary NET, and reviewed radiologic features of extrahepatic biliary NET in previous literature.

Diagnosis of bile duct hepatocellular carcinoma thrombus without obvious intrahepatic mass

AIM:To study the diagnosis of hepatocellular carcinoma(HCC)presenting as bile duct tumor thrombus with no detectable intrahepatic mass.METHODS:Six patients with pathologically proven bile duct HCC thrombi but no intrahepatic mass demonstrated on the preoperative imaging or palpated intrahepatic mass during operative exploration,were collected.Their clinical and imaging data were retrospectively analyzed.The major findings or signs on comprehensive imaging were correlated with the surgical and pathologic findings.RESULTS:Jaundice was the major clinical symptom of the patients.The elevated serum total bilirubin,direct bilirubin and alanine aminotransferase levels were in concordance with obstructive jaundice and the underlying liver disease.Of the 6 patients showing evidence of viral hepatitis,5 were positive for serum alpha fetoprotein and carbohydrate antigen 19-9,and 1 was positive for serum carcinoembryonic antigen.No patient was correctly diagnosed by ultrasound.The main features of patients on comprehensive imaging were filling defects with cup-shaped ends of the bile duct,with large filling defects presenting as casting moulds in the expanded bile duct,hypervascular intraluminal nodules,debris or blood clots in the bile duct.No obvious circular thickening of the bile duct walls was observed.CONCLUSION:Even with no detectable intrahepatic tumor,bile duct HCC thrombus should be considered in patients predisposed to HCC,and some imaging signs are indicative of its diagnosis.

Neuroendocrine carcinoma of the extrahepatic bile duct: A case report

Neuroendocrine carcinoma(NEC) originating from the gastrointestinal hepatobiliary-pancreas is a rare, invasive, and progressive disease, for which the prognosis is extremely poor. The patient was a 72-year-old man referred with complaints of jaundice. He was diagnosed with middle extrahepatic cholangiocarcinoma(cT 4N1M0, c Stage Ⅳ). He underwent a right hepatectomy combined with extrahepatic bile duct and portal vein resection after percutaneous transhepatic portal vein embolization. Microscopic examination showed a large-cell neuroendocrine carcinoma according to the WHO criteria for the clinicopathologic classification of gastroenteropancreatic neuroendocrine tumors. Currently, the patient is receiving combination chemotherapy with cisplatin and etoposide for postoperative multiple liver metastases. Although NEC is difficult to diagnose preoperatively, it should be considered an uncommon alternative diagnosis.

A case of very large intrahepatic bile duct adenoma followed for 7 years

A 70-year-old man was referred to our hospital due to abnormal liver function. A tumor of 92 mm × 61 mm was detected on ultrasound screening of the left liver lobe. Although the tumor was suspected to be intrahepatic bile duct carcinoma, he had chronic heart disease and was unable to undergo surgery. Therefore, he was followed without further testing. No increase in tumor serum markers or tumor size was observed for the subsequent 7 years. We continued to suspect intrahepatic bile duct carcinoma, and we decided to perform a tumor biopsy. Tumor biopsy findings indicated intrahepatic bile duct adenoma(BDA), which is a rare benign epithelial liver tumor typically ranging from 1 mm to20 mm. We herein report a case of very large BDA followed for 7 years.

Intraductal papillary neoplasm of the bile duct accompanying biliary mixed adenoneuroendocrine carcinoma

We present the first case of an intraductal papillary neoplasm of the bile duct(IPNB) accompanying a mixed adenoneuroendocrine carcinoma(MANEC).A 74-yearold woman presented with fever of unknown cause.Laboratory data revealed jaundice and liver injury.Contrast-enhanced computed tomography revealed a 20 mm polypoid tumor in the dilated distal bile duct,which exhibited early enhancement and papillary growth.Upper gastrointestinal endoscopy revealed mucus production from the papilla of Vater,characterized by its protruding and dilated orifice.Endoscopic ultrasonography visualized the polypoid tumor in the distal bile duct,but no invasive region was suggested by diagnostic imaging.Therefore,the initial diagnosis was IPNB.After endoscopic nasobiliary drainage,a pylorus-preserving pancreaticoduodenectomy was performed.Pathological examination of the resected bile duct revealed papillary proliferation of biliary-type cells with nuclear atypia,indicating pancreaticobiliary-type IPNB.In addition,solid portions comprised of tumor cells with characteristic salt-and-pepper nuclei were evident.Immunohistochemistry revealed expression of the neuroendocrine marker synaptophysin in this solid component,diagnosing it as a neuroendocrine tumor(NET).Furthermore,the MIB-1 proliferation index of NET was higher than that of IPNB,and microinvasion of the NET component was found,indicating neuroendocrine carcinoma(NET G3).This unique case of MANEC,comprising IPNB and NET,provides insight into the pathogenesis of biliary NET.

Changing trends of surgical treatment of hilar bile duct cancer:clinical and experimental perspectives

HISTORICAL POINT OF VIEWCarcinoma of the confluence of the hepatic ductshas been thought to be a rare disease until recently.Carcinoma of the large hepatic ducts was rarelydiagnosed correctly premorterned.Because of itsdeeply seated location,resection was once thoughtto be impossible.In 1957,Altemeier reported

Concomitant adenosquamous carcinoma and cystadenocarcinoma of the extrahepatic bile duct: A case report

BACKGROUND Infiltrative adenosquamous carcinoma(ASC) of the extrahepatic bile duct is reported infrequently, which is an unusual variant of the ordinary adenocarcinoma. The simultaneous development of ASC and cystadenocarcinoma in the extrahepatic biliary tree is rare. In addition, the accurate preoperative diagnosis of concomitant carcinoma in the multiple biliary trees at an early stage is often difficult. Thus, awareness of the risk of the multiplicity of biliary tumors is perhaps the most important factor in identifying these cases.CASE SUMMARY Here, we report a case of a 63-year-old female with jaundice, who was referred to Shuguang Hospital because of abdominal pain for 1 mo. An abdominal contrastenhanced computed tomography revealed a type I choledochal cyst and intraluminal masses suggestive of adenoma of the common bile duct. In addition,a preoperative diagnosis of a concomitant Klatskin tumor and type I choledochal cyst was made. The patient underwent anti-inflammatory therapy, followed by radical surgery due to hilar cholangiocarcinoma and resection of the choledochal cyst. Examination of the surgical specimen revealed a papillary tumor of the common bile duct, which arose from the malignant transformation of a preexisting cystadenoma. Histologic examination confirmed a special type of cholangiocarcinoma; the tumor in the hilar bile duct was an ASC, whereas the tumor in the common bile duct was a moderately differentiated cystadenocarcinoma. The patient showed rapid deterioration 8 mo after surgery.CONCLUSION Although concomitant ASC and cystadenocarcinoma of the extrahepatic bile duct is difficult to diagnose before surgery, and the prognosis is poor after surgery,surgical resection is still the preferred treatment.