Mirizzi syndrome secondary to an adenoma of the cystic duct

Bile duct adenomas are uncommon lesions that can cause obstructive jaundice. We report the unusual case of a 54-year-old man who developed Mirizzi syndrome secondary to a bile duct papillary adenoma located in the cystic duct remnant. A case report is presented, together with a review of extrahepatic bile duct adenomas published in the English-language literature, with special attention directed toward the clinical manifestations, locations, and prognosis of these tumors. Bile duct adenomas are very rare tumors. Although cholangiography can detect many of these lesions, few cases were correctly diagnosed preoperatively. Most lesions were located in the distal common bile duct or at the ampulla of Vater. Pathologic examination often revealed foci of carcinoma in situ, dysplasia, or atypia. Local resection was performed in most cases. There were no previous case reports of extrinsic common bile duct obstruction caused by tumors within the cystic duct. We describe here a very rare, acalculous variant of Mirizzi syndrome secondary to a solitary papillary adenoma of the cystic duct. In general, bile duct adenomas are uncommon lesions that are difficult to diagnoses preoperatively. These tumors usually present with jaundice secondary to intraluminal biliary obstruction. These lesions are premalignant and should be managed by complete surgical resection.     

Endoscopic biliary imaging and clinicopathological features of cystic duct cancer

Background Cystic duct cancer fulfilling Farrar’s criteria is relatively rare, but tumors whose origin is estimated to be in the cystic duct exist. The clinical features of such “broadly defined” cystic duct cancer have not been clarified. Methods The endoscopic retrograde cholangiography (ERC) findings, intraductal ultrasonography (IDUS) findings, histological findings, and prognoses of 11 cases of cystic duct cancers resected at our institution (group C) were retrospectively analyzed. As a control group, 55 cases of middle or lower bile duct cancer (group B) were used (in 20 of the 55 cases of group B, tumors extended to the cystic duct intraluminally (group B-C (+)). Results (1) ERC findings of group C as compared with those of group B-C (+) were as follows: (a) unilateral bile duct narrowing (spoon-like appearance): 55% versus 5% (P < 0.01); (b) bilateral bile duct narrowing (apple-core-like appearance): 27% versus 95% (P < 0.001). (2) IDUS was unable to visualize the cysticocholedochal junction (negative “confluence sign”) more often in group C (67%) than in group B-C (+) (13%) (P < 0.01). (3) Histologically, tumors extended to the gallbladder and the bile duct in 36% and 91% of the cases in group C, respectively. (4) The median survival time of the two groups was 21 and 28 months, respectively. Conclusions Cystic duct cancers frequently extended to the bile duct. The spoon-like appearance by ERC and the negative confluence sign by IDUS were characteristic findings.     

Double cancer of the cystic duct and gallbladder associated with low junction of the cystic duct

We report a case of double cancer of the cystic duct and gallbladder associated with low junction of the cystic duct. A 73-year-old woman was admitted to the hospital complaining of upper abdominal pain. Endoscopic retrograde cholangiography showed a stenotic lesion in the lower common bile duct and no visualization of the cystic duct or gallbladder. Enhanced computed tomography revealed a heterogeneously enhanced tumorous lesion around the lower bile duct in the pancreatic head. A diagnosis of cancer arising from the cystic duct that entered the lower part of the common hepatic duct was made by intraductal ultrasonography, which showed an intraluminal protruding lesion in the cystic duct. Isolated gallbladder cancer was also diagnosed, by abdominal computed tomography. She underwent pancreaticoduodenectomy with dissection of regional lymph nodes. Histological examination revealed moderately differentiated adenocarcinoma of the cystic duct and well-differentiated adenocarcinoma of the gallbladder. Double cancer of the cystic duct and gallbladder is extremely rare, and this case also suggests a relationship between a low junction of the cystic duct and neoplasm in the biliary tract.     

Intraductal papillary neoplasm of the bile duct extending superficially from the intrahepatic to extrahepatic bile duct

Intraductal papillary neoplasm of the bile duct (IPNB) or liver is a recently noted rare disease, and its pathogenesis remains unclear. Here we present a case of IPNB with an interesting morphology, which was treated by resection of the right hemiliver and extrahepatic bile duct. A 79-year-old woman was found to have a high alkaline phosphatase level and slight dilatation of the right intrahepatic bile duct on imaging studies. The right intrahepatic bile duct became dilated over a 2-year period; however, no solid mass could be detected, and tumor markers were not elevated. Hepatic resection was scheduled because a mucin-producing bile duct carcinoma of the liver was suspected. A right hemihepatectomy was conducted, and the extrahepatic bile duct was also resected after malignant cells were found in the surgical stump of the right bile duct and in the bile itself. Macroscopically, diffuse dilatation of the intrahepatic bile duct was noted, but no solid component or mucin within the duct was found. Histopathological findings revealed carcinoma in situ, IPNB, in the majority of intrahepatic bile ducts, with no lymph node metastasis, and it extended continuously to the epithelium of the common bile duct. No tumor recurrence or biliary dilatation was observed at follow-up 2 years after surgery. It is important to consider malignancy in the presence of a dilated bile duct and in the absence of any cause of occlusion. Complete resection of IPNB results in a good prognosis and no recurrence.     

腹腔镜胆囊切除术1475例胆囊动脉胆囊管的解剖与处理

目的:报告腹腔镜胆囊切除术中胆囊动脉及胆囊管的解剖观察。方法:1991—1993年行腹腔镜胆囊切除术1475例,手术认真解剖了胆囊动脉和并用钛夹分别进行钳闭处理。结果:84.9％为主干型胆囊动脉,15.1％为多支型胆囊动脉、胆囊动脉缺如或细小,肝迷走胆囊动脉等变异。90.9％的胆囊管直径0.3—0.4cm,长1—3.5cm。因胆囊炎症的严重程度导致胆囊管纤维化闭锁或增粗多见。短胆囊管76例。因误认致肝外胆管横断伤3例,胆囊动脉后支出血止血钳闭部分肝总管1例,胆囊管残端瘘1例,变异胆囊动脉术后出血2例。术后并发心律失常、肺部感染,21d死于多器官功能衰竭1例(0.1％)。治愈1474例(99.9％)。结论:腹腔镜胆囊切除术治疗胆石症是-安全的方法,术中仔细解剖胆囊动脉和胆囊管极为重要。     

胆囊管癌伴右副肝管直接汇入胆囊管1例报告

正胆道变异的类型繁多,包括胆囊管与肝总管并行低位汇合,胆囊管汇入右肝管、副肝管等~([1])。其中副肝管又叫Luschka胆管或迷走胆管,是胚胎期肝十二指肠发育不全的常见畸形,直接连接胆囊和肝内胆管通道,常开口于胆总管,其次是右肝管和胆囊管~([2])。本文报道1例较大右副肝管直接汇入胆囊管,并伴有胆囊管癌的罕见病例。1病例资料患者男性,63岁,因"间断性上腹部胀痛不适2年,加重伴     

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.     

Multiple carcinoid tumors of the gallbladder and cystic duct

A very rare case of multiple carcinoid tumors of the gallbladder and cystic duct is reported in a 77-year-old woman. Cholecystectomy and resection of the common bile duct, with regional lymph node dissection, were performed. The tumors consisted of a large mass (30×20×15mm) in the neck of the gallbladder and three small lesions in the cystic duct. Histologically, the tumor cells were arranged in solid nests, and showed many mitoses. Histochemical studies revealed argyrophilic and argentaffinic staining. Immunohistochemically, the tumor cells were positive for serotonin, chromogranin A, Leu 7, synaptophysin, and neuron-specific enolase. Thus, the present case was diagnosed as midguttype carcinoids.     

Iatrogenic bile duct injuries from biliary tract surgery

BACKGROUND:Cholecystectomy is the most commonly performed procedure in general surgery.However,bile duct injury is a rare but still one of the most common complications.These injuries sometimes present variably after primary surgery.Timely detection and appropriate management decrease the morbidity and mortality of the operation. METHODS:Five cases of iatrogenic bile duct injury(IBDI) were managed at the Department of Surgery,First Affiliated Hospital,Xi'an Jiaotong University.All the cases who underwent both open and laparoscopic cholecystectomy had persistent injury to the biliary tract and were treated accordingly. RESULTS:Recovery of the patients was uneventful.All patients were followed-up at the surgical outpatient department for six months to three years.So far the patients have shown good recovery. CONCLUSIONS:In cases of IBDI it is necessary to perform the operation under the supervision of an experienced surgeon who is specialized in the repair of bile duct injuries,and it is also necessary to detect and treat the injury as soon as possible to obtain a satisfactory outcome.     

High-grade dysplasia of the cystic duct margin in the absence of malignancy after cholecystectomy

BackgroundA total of 750 000 cholecystectomies are performed annually in the USA. No data exist on patients with microscopic high-grade dysplasia at the cystic duct margin and the associated incidence of cholangiocarcinoma.MethodsPathology reports for 1992–2010 were reviewed for patients with high-grade dysplasia of the cystic duct margin in the absence of invasive gallbladder cancer. Clinical data were obtained from chart review.ResultsFive patients with high-grade dysplasia at the cystic duct margin without evidence of malignancy were identified. Radiologic imaging was abnormal in two patients. The cystic duct stump was abnormally dilated in both patients and one patient had an enlarged portacaval lymph node. All five patients underwent exploration and resection of either the cystic duct stump or the bile duct. Specimens in four of the patients showed no evidence of malignancy or dysplasia. One patient was found to have a node-positive adenocarcinoma of the cystic duct.ConclusionsHigh-grade dysplasia at the cystic duct margin without evidence of invasive gallbladder cancer is rare. Patients with this finding should undergo cross-sectional imaging and a diagnosis of an underlying cholangiocarcinoma should be considered, especially if imaging reveals any abnormalities.     

Advanced bile duct carcinoma in a 15-year-old patient with pancreaticobiliary maljunction and congenital biliary cystic disease

We report a case of advanced bile duct carcinoma arising in a 15-year-old female with pancreaticobiliary maljunction and congenital biliary cystic disease. Pancreaticoduodenectomy and partial resection of the liver was performed. Surgical and histopathological findings indicated advanced tubular adenocarcinoma, classified as final stage IVb according to the General rules for surgical and pathological studies on cancer of the biliary tract proposed by the Japanese Society of Biliary Surgery, 5th edition, and stage IV according to the American Joint Committee on Cancer (AJCC)/International Union Against Cancer (UICC), 6th edition. She underwent chemotherapy with gemcitabine HCl after discharge. She died of cachexia 14 months after the surgery. Although it is well known that biliary malignancies arise frequently in patients with pancreaticobiliary maljunction, it is uncommon for advanced bile duct carcinoma to occur in a 15-year-old female. We should pay attention to the possibility of biliary malignancy in patients with pancreaticobiliary maljunction and congenital biliary cystic disease, even when the patients are juveniles.     

内镜逆行胰胆管造影诊断胆囊管狭长弯曲与外科治疗

目的 探讨内镜逆行胰胆管造影（ＥＲＣＰ）诊断胆囊管狭长弯曲和外科治疗效果。方法 随访分析１７例患者的主要临床表现、病因、诊断方法和手术疗效。１７例患者术前均行内镜逆行胰胆管造影检查,术中行胆道造影检查,手术方式为胆囊切除术。结果 经２～５年的随访,效果满意。结论 胆囊管狭长弯曲是临床并不少见的胆道疾病,ＥＲＣＰ可提高检出率,行胆囊切除术疗效好。     

Carcinoma of the cystic duct associated with pancreaticobiliary maljunction

Received: February 4, 2000 / Accepted: May 26, 2000     

经内镜逆行胰胆管造影在肝外胆管腺瘤诊治中的应用

目的探讨肝外胆管腺瘤的临床特征及经内镜逆行胰胆管造影(ERCP)在其诊断及治疗中的作用。方法回顾性分析2010年1月-2016年6月苏北人民医院消化内科收治的7例肝外胆管腺瘤患者的临床表现、影像学特点、ERCP治疗及随访情况。结果 7例患者入院排除手术禁忌后,择期行ERCP,术中胆管造影见胆管不同部位显影中断,均予以导丝引导下细胞刷检及超细活检钳胆管内活组织检查,病理提示胆管腺瘤,6例患者选择ERCP治疗,1例患者ERCP术后行胆总管空肠Roux-en-Y吻合术。术后随访6~12个月,6例患者恢复良好,1例患者术后2个月并发胆管炎。结论 ERCP作为微创检查手段,有助于明确病因和判断病情。当患者存在外科手术禁忌或暂时不愿选择外科手术时,ERCP可作为一种姑息性治疗手段,能很好的缓解患者临床症状。     

Malignant carcinoid tumor of the cystic duct:a rare cause of bile duct obstruction

BACKGROUND:Carcinoid tumors of the extrahepatic biliary tree are extremely rare malignancies,accounting for 0.2%-2%of all gastrointestinal carcinoid tumors,while carcinoids of the cystic duct are an uncommon entity and an extremely unusual cause of bile duct obstruction.METHODS:After an extensive literature review,we retrospectively analysed 61 cases of carcinoid tumor of the biliary tree as well as one additional case of a 60-yearold female with symptoms and laboratory/imaging examination findings compatib...     

A case of double cystic duct with cholecystolithiasis treated by laparoscopy

A case of cholecystolithiasis with double cystic duct treated successfully by laparoscopic surgery is reported. The patient was a 50-year-old female who presented with abdominal pain in the right upper quadrant. On admission, extracorporeal ultrasonography (US) revealed a hyperechoic area accompanied by an obscure acoustic shadow in the gallbladder. Endoscopic retrograde cholangiopancreatography (ERCP) revealed two cystic ducts that led separately from the same cluster of the gallbladder. After preoperative examination around the biliary tree, we determined that laparoscopic cholecystectomy was the treatment of choice. Intraoperative color Doppler US was useful for distinguishing the cystic duct from vessels. An ultrasound aspirator (UA) was also extraordinarily useful for skeltonizing the cystic ducts and the cystic artery. The postoperative course was not eventful. Our findings suggest that laparoscopic cholecystectomy, using an UA, is indicated in patients with an anomalous arrangement of the biliary system, since the use of the UA provides a clear delineation of the anatomy of Calot's triangle.     

Recurrent carcinoma of cystic duct remnant with subcutaneous implantation in abdominal wall

A case of recurrent carcinoma of the cystic duct remnant invading the common bile duct and portal vein with subcutaneous implantation of the abdominal wall is presented. The patient was a 55-year-old woman with an abdominal wall tumor at the site of the surgical scar of a cholecystectomy, performed at a local hospital 5 years ago for symptomatic cholelithiasis. The diagnosis was made by incisional biopsy of the tumor, computed tomography, percutaneous transhepatic cholangiography, and angiography. She underwent extended right hepatic lobectomy with en bloc resection of the caudate lobe, extrahepatic bile duct, and portal vein. The abdominal wall tumor was resected concomitantly. Histological examination showed that both the recurrent carcinoma of the cystic duct remnant and the abdominal wall implantation were moderately differentiated adenocarcinoma. This recurrence probably could have been prevented if both the macroscopic and microscopic examinations of the resected specimen had been precisely carried out after the previous cholecystectomy and the primary carcinoma identified and treated at that time.     

Frequent activation of mitogen-activated protein kinase relative to Akt in extrahepatic biliary tract cancer

Background Lack of effective adjuvant therapy against advanced extrahepatic biliary tract carcinoma (BTC) requires that new therapeutic methods, such as molecular targeted therapy, be developed. The mitogen-activated protein kinase (MAPK) and Akt signaling pathways, which activate cell proliferation and suppress apoptosis, respectively, may function as important targets for such therapies. The aim of this study was to examine the expression patterns of phosphorylated MAPK (p-MAPK) and phosphorylated Akt (p-Akt) proteins in BTC cell lines and clinical specimens. Methods Expression of p-MAPK and p-Akt proteins in four human BTC cell lines and in frozen sections of 20 advanced extrahepatic BTC specimens was analyzed by Western blotting. Thirty formalin-fixed BTC specimens were immunohistochemically stained for p-MAPK and p-Akt using labeled streptavidin–biotin conjugates. Results Expression of p-MAPK was observed in three of four (75%) BTC cell lines, whereas no expression of p-Akt was observed. Twenty-three of 30 formalin-fixed specimens stained positive for p-MAPK (77%), whereas only 47% stained positively for p-Akt. Expression of p-MAPK relative to that of p-Akt was also seen more frequently in the frozen specimens. Conclusions The results of this study suggest that MAPK is activated more frequently than Akt in extrahepatic biliary tract carcinoma.     

Carcinosarcoma of the extrahepatic bile duct

A rare case of a carcinosarcoma of the extrahepatic bile duct demonstrating interesting features is described. A 75-year-old woman with a history of choledocholithotomy presented with acute obstructive suppurative cholangitis. Ultrasonography and computed tomography showed a thickened choledochal wall, with calcification. Percutaneous transhepatic and endoscopic retrograde cholangiography revealed a round filling defect accompanied by an irregular obstruction in the common bile duct. Carcinosarcoma was diagnosed from a protruding lesion in the common bile duct obtained by intraoperative frozen sectioning, and pylorus-preserving pancreatoduodenectomy was performed. Histological examination by light microscopy showed a transition between the carcinomatous and sarcomatous components and positive immunoreactivity for epithelial markers in the sarcomatous component. The patient died of a local recurrence 2 years after the surgery. Polypoid growth and ossification in the tumor could be representative features of carcinosarcoma of the extrahepatic bile duct.