Carcinoid Tumors of the Common Bile Duct: Report of Two Cases

We report two cases of carcinoid tumors of the common bile duct. The first patient was a 65-year-old woman in whom a carcinoid tumor of the distal bile duct was incidentally found during an open cholecystectomy for cholecystitis. The second patient was a 27-year-old man in whom a distal common bile duct carcinoid was incidentally found during orthotopic liver transplantation for sclerosing cholangitis and multiple biliary strictures. There are few reports of carcinoid tumors of the extrahepatic ducts, and a brief review of the relevant literature is discussed following these case reports. Received: April 22, 2002 / Accepted: November 19, 2002 RID="*" ID="*" Reprint requests to: D. K. Imagawa     

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.     

Carcinoid tmour of the common bile duct: report of a case and a review of the literature

Carcinoid tumours of the common bile duct are extremely rare lesions. In this article we report a case with an extrahepatic bile duct carcinoid tumour. A 40-year-old woman suffered from biliary colic and jaundice. Pre-operative computed tomography demonstrated a tumour in the biliary tract. At laparotomy there was a tumour invading the common bile duct. Common bile duct resection was performed. Carcinoid tumour of the common bile duct was diagnosed histopathologically. For extrahepatic bile duct carcinoid tumours surgical resection is the only treatment modality that offers a chance to provide a cure and prolonged disease-free survival. The favourable histopathological and biological features of these tumours encouraged the surgeons to use more aggressive approaches for advanced disease.     

Carcinoid tumours of the bile ducts

Carcinoid tumors of the extrahepatic bile ducts are extremely rare and account for only 0.2-2% of all gastrointestinal endocrine neoplasms. They are particularly difficult to diagnose preoperatively and distinguish from cholangiocarcinoma. We report the case of a 52-year-old patient with a carcinoid tumour of the common bile duct presenting with obstructive jaundice. Laboratory and imaging studies (US, CT, MRI) showed an obstructive lesion measuring 2 cm in diameter in the common bile duct. A biliary stent was inserted initially to decompress the bile ducts. Brushing was negative for malignant cells. With a suspicion of malignancy (confirmed intraoperatively), the patient underwent radical resection of the extrahepatic duct, portal lymphadenectomy and a Roux-en-Y hepaticojejunostomy. The pathology examination revealed a well-differentiated neuroendocrine tumour of the common hepatic duct. The tumour cells were immunopositive for chromogranin A and synoptophysin. There was no evidence of metastases in the nodes removed and negative surgical margins were achieved. The patient is disease-free after a follow up of 3 months, and levels of chromogranin A and neuron-specific enolase are normal. Local and systemic aggressiveness of these tumours is rare. Radical surgery offers the only chance of a cure and has to be considered whenever possible.     

Carcinoid of the extra-hepatic bile duct: a case report with long-term follow-up and review of literature

Extrahepatic bile duct tumors, 80 per cent of which are adenocarcinomas, are rare neoplasms accounting for less than two per cent of all cancers. Carcinoid tumor of the extrahepatic bile ducts is a reportable lesion, with only approximately 50 cases described in the literature since 1959. We present a case of a primary extrahepatic bile duct carcinoid tumor resected for cure with the longest reported follow-up time (11 years) after surgery. We also summarize the existing literature with regard to this rare tumor. Our case lends strong support to the notion that extrahepatic biliary carcinoids are generally indolent lesions that, if aggressively resected, can result in excellent long-term survival. Complete excision with clear margins seems to provide the best chance of obtaining long-term survival and cure.     

The reliability and clinical limitations of sonographic scanning of the biliary ducts.

Sonographic scanning of the biliary ducts has been successfully used as a screening test to distinguish between patients with surgical and medical jaundice, with an accuracy of 90%. However, there is no consensus in the literature on what numerically defines a dilated biliary duct. To clarify this problem a prospective study of 102 consecutive patients was initiated to determine the sonographic size range of bile ducts in patients with and without extrahepatic ductal obstruction. The ultrasonic measurements were compared with direct measurements of the common bile duct, at surgery. The extrahepatic ductal system was visualized sonographically in 62% of the patients, while the intrahepatic ducts were found in 81% of the population. Direct measurements at operation agreed with the ultrasonic measurements in 84% of the patients. Analysis of the size range of the biliary ducts in patients with and without extrahepatic obstruction, by chi square analysis and the Student's t-test, allowed the following guidelines to be established. Extrahepatic bile duct obstruction was present if the extrahepatic bile ducts was 1 cm or wider (p less than 0.001) or if the intrahepatic bile duct was in excess of 0.5 cm (p less than 0.001). Similarly if the extrahepatic bile duct measured less than 0.8 cm sonographically, and the intrahepatic bile duct was 0.4 cm or less than bile duct, obstruction was not present (p less than 0.001).     

The etiology of "white bile" in the biliary tree

"White bile" is the colorless fluid occasionally found in occluded biliary systems. The absence of pigments in this "bile" was not satisfactorily explained. The objectives of this study were to assess its etiology. In dogs, "white bile" developed whenever both the common bile duct and the cystic duct were ligated. In comparison, dark green ("black") bile occurred when only the common bile duct was ligated leaving the gallbladder in communication with the obstructed ducts. The pressure in extrahepatic ducts containing "white bile" was significantly higher than in those filled with "black bile." Flow in the extrahepatic ducts was assessed by the aid of radioiodinated human serum albumin (RIHSA). When "black bile" was present, the direction of flow was from the extrahepatic ducts into the gallbladder. Whenever "white bile" developed, a reverse flow from the extrahepatic ducts into the liver was observed. Thus, the role of the gallbladder appears to be decompression of the biliary system allowing bile flow from the liver even in obstruction. In the absence of the gallbladder water absorption activity, the colorless secretion of the bile ducts seems to "back wash" into the liver and replace the bile present in the ducts at the time of occlusion.     

Clinico-pathological studies on a transitional type between extrahepatic biliary atresia and paucity of the interlobular bile ducts

Among the neonatal and infantile cases of obstructive jaundice seen at Niigata and Yamagata University Hospitals between 1976 and 1990, extrahepatic bile ducts were visualized in 19 cases by either preoperative endoscopic retrograde cholangiopancreaticography (ERCP) or intraoperative cholangiography. Neonatal hepatitis was diagnosed in 3 of these cases by clear images of the bile duct system extending from the common bile duct to the intrahepatic bile duct. In 7 cases, the common bile duct was able to be seen, while the common hepatic duct was only slightly visualized. Four of these 7 cases were consistent with paucity of the interlobular bile ducts (PILBD) based on hepatic histology, while the remaining 3 showed fibrosis, bile ductular proliferation, and many bile plugs in the bile ductuli of the portal areas, concurrent with histological changes in extrahepatic biliary atresia (EHBA), not PILBD. In 9 cases, only the common bile duct was visualized while the common hepatic duct was not seen, 7 of these 9 cases being consistent with type III-al EHBA. In 2 cases, neither fibrosis nor proliferation of the bile ductuli was observed in the portal areas, and portal areas without any bile ductuli were also seen, in accordance with findings for PILBD. Three cases which showed similar hepatic histological findings to EHBA despite the presence of patent extrahepatic bile ducts, and 2 cases which had obstructed extrahepatic bile ducts and hepatic histological findings similar to PILBD, were thought to be of a transitional type between EHBA and PILBD. Thus, it is postulated that the entire bile duct system covering hepatocytes to extrahepatic bile ducts is affected by a certain factor, but the expression of individual disease types may be dependent upon the location of the site most seriously affected.     

Teratoma arising from anomalous common bile ducts: a case report

Teratoma arising from extrahepatic common ducts is very rare entity. The authors found 2 teratoma cases originating from common bile duct in the literature. As a third case, the authors report on a 4-month-old girl with benign cystic teratoma arising from distal common hepatic bile duct and with anomalous common bile ducts. Surgical management of the patient also is discussed.     

胆管类癌32例并文献复习

目的探讨肝外胆管类癌的临床特征和临床诊治。方法报告2例胆总管类癌并结合文献报道30例;对32例肝外胆管类癌进行分析。结果肝外胆管类癌临床少见,32例中女性23例,男性9例,发病年龄平均为49岁(19～79)岁。临床表现梗阻黄疸最为常见,占55%。肿瘤局限于胆管内为71%,诊断时己有远处转移的为29%,其肿瘤局限于胆管内病例3年存活率为100%。结论肝外胆管类癌与胆管腺癌不同,其发病年龄年轻(平均为49岁),并好发于女性。肿瘤的局部浸润为主,很少发生远处转移,根治切除后可获长期生存,对胆管类癌应行积极的根治切除。     

腹腔镜胆囊切除术中肝外胆道解剖异常的防范

目的探讨腹腔镜胆囊切除术（1aparoscopic cholecystectomy，LC）中肝外胆道异常的诊断及处理。方法1999年10月～2008年6月1216例LC中，发现15例（1．2％）胆道解剖异常。3例胆囊管异常粗、短，开口在左右肝管汇合部；1例胆囊颈部结石嵌顿，胆总管较细，向上牵拉胆囊使胆总管走行移位；1例胆囊管与肝总管并行后低位开口，1例胆囊管在胆总管右侧回旋扭曲，开口于右肝管侧壁，2例胆囊壶腹部粘连严重，覆盖于胆总管及肝总管前方；3例在胆床附近见迷走胆管走行；3例在分离胆囊管时发现右后肝管开口于肝总管；1例Mirizzi综合征解剖不清。仔细分离，丝线结扎或上钛夹处理，解剖不清者中转开腹。结果13例顺利完成LC；2例（13．3％）中转开腹，其中1例副右肝管损伤，1例Mirizzi综合征。无腹腔内出血、腹腔感染、肠道损伤及死亡等严重并发症。15例随访3个月～4年，其中〉1年11例，无胆道狭窄及残余结石。结论LC术中精细解剖胆囊三角，确切辨认各管道关系，是预防胆道异常情况下肝外胆道损伤的关键。     

豚鼠肝外胆道不同部位组织缺血-再灌注损伤的比较观察

目的比较肝外胆道不同部位组织缺血-再灌注损伤的特点及差异。方法制成豚鼠肝外胆道缺血-再灌注模型,观察不同部位组织缺血30min再灌注60min后,上皮细胞线粒体平均体积（     

Appendix Carcinoid Found Incidentally During Excision of a Choledochal Cyst: Report of a Case

We report the case of a 13-year-old girl in whom an appendix carcinoid was found incidentally during excision of a choledochal cyst. Although incidental carcinoid tumors of the extrahepatic bile ducts have been reported, to the best of our knowledge this is the first published case of a choledochal cyst associated with an appendix carcinoid. We present this case to highlight the importance of conducting a thorough exploration of all quadrants of the abdomen before closure.     

Adenomyoma of the Common Hepatic Duct Mimicking Bile Duct Cancer: Report of a Case

We report an unusual case of adenomyoma of the common hepatic duct mimicking bile duct cancer. A 50-year-old woman was referred to our hospital for the investigation of general fatigue. Laboratory data showed abnormal liver test results and computed tomography showed a mass lesion in the hepatic hilum and dilatation of the intrahepatic bile ducts. These findings led to a preoperative diagnosis of hilar bile duct carcinoma, and we performed a left lobectomy with resection of the extrahepatic bile duct. Macroscopically, an elevated lesion was found in the common hepatic duct, which was confirmed histologically to be an adenomyoma. Bile duct strictures are rarely caused by benign tumors of the biliary tract, such as adenomyoma. Surgical resection of the bile duct should be considered for all bile duct strictures because it is often difficult to differentiate malignant from benign lesions in this location preoperatively, and malignant cells may be present in the lesion.     

Association of extrahepatic bile duct duplication with pancreaticobiliary maljunction and congenital biliary dilatation in children: a case report and literature review

We herein report a case of cystic-type congenital biliary dilatation (CBD) in whom an extremely rare anomalous duplication of the common bile duct and pancreaticobiliary maljunction were diagnosed intraoperatively by meticulous surgical manipulations via conventional open surgery. By performing a dissection at the outer epicholedochal layer of the cyst, a thin cord-like structure shown to be the distal part of the common bile duct was identified. A further exploration revealed that the most distal (extra- and intrapancreatic) part of the common bile duct was duplicated, and each branch of the duct was connected to the main and accessory pancreatic ducts. The experience with our case and a literature review showed that extrahepatic bile duct duplication is generally associated with pancreaticobiliary maljunction and CBD. We conclude that an extremely careful exploration with delicate and meticulous surgical manipulation is essential to identify these morphological anomalies and prevent intraoperative and postoperative complications of CBD, such as pancreatic duct injury or pancreatitis.     

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.     

Choledochal cyst as a complex malformation of the intra and extrahepatic bile ducts

The authors present the case of a 64 years woman with a choledochal cyst along with a complex malformation of both intra and extrahepatic bile ducts. The patient was admitted with a diagnosis of acute and underestimated cholecystitis, which was in fact a real acute severe suppurated angiocolitis located at the cystic cavity level along with severe hepato-renal failure. As long as the choledochal cyst evolves as a stand-alone entity, it can frequently be associated with other malformations of the bile ducts--such as choledochal duct stenosis or abnormal connection of the common hepatic duct with pancreatic ducts. The main dilatation was engulfing the whole common bile duct with a fusiform aspect, and the cranial end is opening into a long hepatic duct from which were emerging several biliary segments branches, for both hepatic lobes. This aspect of a multistage convergence with four branches which is opening into a long hepatic bile duct and then in the choledochal cyst, represents an unusual malformation which does not respect the rules described by former published authors.     

Undifferentiated carcinoma of the common bile duct with intraductal tumor thrombi: report of a case

We report a case of undifferentiated carcinoma of the common bile duct with intraductal tumor thrombi. A 73-year-old man presented with general malaise. Abdominal computed tomography and magnetic resonance imaging revealed a mass in the distal common bile duct, accompanied by dilatation of the intra- and extrahepatic bile ducts. The patient underwent pancreaticoduodenectomy with regional lymphadenectomy. Gross examination revealed that the distal common bile duct was obstructed by an elastic hard mass, 3.2 × 2.6 cm, accompanied by intraductal tumor thrombi. Microscopically, the nodule was well defined and composed of atypical large tumor cells with bizarre nuclei and little cytoplasm. Immunohistochemically, the tumor cells were diffusely positive for cytokeratin-7 and CAM5.2, but negative for CD56, chromogranin A, and synaptophysin. Thus, a histological diagnosis of undifferentiated carcinoma of the common bile duct was made. The patient recovered uneventfully and has remained free of any signs of recurrence for 18 months since the operation. Undifferentiated carcinomas of the extrahepatic bile duct can be detected early, with the chance of a good prognosis; however, because their biologic growth behavior is still considered aggressive, careful observation after surgery and the initiation of multidisciplinary treatment against recurrence are necessary.     

Magnetic resonance cholangiography for the diagnosis of biliary atresia

Purpose: The aim of this study was to evaluate the usefulness of magnetic resonance cholangiography (MRC) for the diagnosis of biliary atresia in infantile cholestatic jaundice. Methods: Forty-seven consecutive infants with cholestatic jaundice underwent single-shot MRC. The diagnosis of biliary atresia was made by MRC based on the nonvisualization of extrahepatic bile ducts and excluded on the basis of the complete visualization of extrahepatic bile ducts. The final diagnosis of biliary atresia (BA group, n = 23) or nonbiliary atresia (NBA group, n = 24) was established by operation or clinical follow-up until the jaundice resolved. Results: The extrahepatic bile ducts including the gallbladder, the cystic duct, the common bile duct, and the common hepatic duct were visualized in 23 of the 24 infants of the NBA group. The extrahepatic bile ducts, except the gallbladder, were not depicted in any infant of the BA group. MRC had an accuracy of 98%, sensitivity of 100% and specificity of 96%, for diagnosis of biliary atresia as the cause of infantile cholestatic jaundice. Conclusions: MRC is a very reliable noninvasive imaging modality for the diagnosis of biliary atresia. In infants with cholestatic jaundice and considered for exploratory laparotomy, MRC is recommended to avoid unnecessary surgery.