老年肝外胆管梗阻85例治疗分析

目的：探讨老年性肝外胆管梗阻的治疗。方法：85例老年胆管梗阻中良性疾病引起的梗阻为59例，主要是由于胆管结石嵌顿MIRRIZI‘S综合征；恶性肿瘤引起的梗阻为26例，主要为壶腹部周围癌，肝门区肿瘤等。手术分别采取胆囊切除术、胆总管切开取石＋T管引流术、胰十二指肠切除术、腹部探查＋内外引流术以及ERCP下行置管内支撑术后引流和ENBD等。结果：良性疾病引起的梗阻中仅1例术后死于ARDS，恶性肿瘤 引起的梗阻者中有11例术后死于肝肾综合征、ARDS和DIC等。结论；手术仍是老年肝外胆管梗阻的首选方法。老年患者合并其它脏器病变，病情较重者如先行胆管引流，能有效降低病死率及延长生命。     

Small cell carcinoma of the extrahepatic bile duct: case report and immunohistochemical analysis

A small cell carcinoma of the extrahepatic bile duct in a 75-year-old Japanese man is reported. The patient suffered from obstructive jaundice, and percutaneous transhepatic cholangiography-drainage (PTCD) revealed a massive lesion in the lower common bile duct. Because it was diagnosed as a malignant tumor, pancreaticoduodenectomy was performed. A nodular infiltrating tumor measuring 4.5 x 3.0 x 2.0 cm was located in the intrapancreatic portion of the extrahepatic bile duct. Histologically, the tumor was composed of a dense proliferation of small atypical cells with a little region of high-grade dysplasia in the adjacent epithelium of the common bile duct. Tumor cells were immunoreactive to neuroendocrine markers such as chromogranin A, synaptophysin, CD56, and Leu7. Although carcinoma cells invaded into pancreas and duodenum, there were no histological findings that indicated the carcinoma arose from the mucosa of either the pancreatic duct or duodenum. These results indicated that the tumor was a small cell carcinoma derived from the epithelium of the extrahepatic bile duct; a rare neoplasm with only a few cases reported. A few neuroendocrine cells were recognized in the adjacent epithelium of the extrahepatic bile duct, suggesting that the tumor cells might be derived from them. Using immunohistochemical examination, no p53 abnormality was found. Tumor cells showed positive nuclear staining for p16, while negative for cyclin D1, suggesting that functional retinoblastoma protein (pRB) might be lost in the p16/pRB pathway, as in small cell lung cancer.     

Cystic duct and Heister's "valves"

The anatomy and physiology of the cystic duct have been relatively neglected by anatomists and the function of the spiral mucosal folds or "valves" of Heister, first described in 1732, remains obscure. The gross and microscopic anatomy of the cystic duct is reviewed together with results from laboratory investigations into the function of the cystic duct and its spirally arranged folds. The duct and spiral folds contain muscle fibers responsive to pharmacologic, hormonal, and neural stimuli. There is, however, no convincing evidence of a discrete muscular sphincter within the duct. Although the cystic duct is unlikely to play a major role in gallbladder filling and emptying, it appears to function as more than a passive conduit. Coordinated, graded muscular activity in the cystic duct in response to hormonal and neural stimuli may facilitate gallbladder emptying. The principal function of the internal spiral folds that are found in man and other animals may be to preserve patency of this narrow, tortuous tube rather than to regulate bile flow.     

Expression of alpha-amylase isoenzymes and trypsin by the proliferating epithelium of large intrahepatic bile ducts and intrahepatic peribiliary glands in hepatolithiasis

The expression of alpha-amylase isoenzymes (pancreatic and salivary) and trypsin by the epithelium of large intrahepatic bile ducts and peribiliary glands was examined immunohistochemically in hepatolithiasis ( n = 22), extrahepatic biliary obstruction ( n = 20) and normal liver ( n = 22). Hepatolithiasis was associated with marked proliferation of bile duct cells and peribiliary glands. Expression of pancreatic and salivary amylase was observed in the proliferating bile duct cells and peribiliary glands of all livers, and trypsin was found in 68% of the livers. In extrahepatic biliary obstruction, proliferation of the biliary epithelium was less marked, but expression of amylase isoenzymes was observed in all livers and trypsin was found in 50%. All normal livers showed expression of amylase isoenzymes in large intrahepatic bile ducts, septal bile ducts and peribiliary glands, and trypsin was found in 73%. The density of enzyme-containing acini was highest in hepatolithiasis, intermediate in extrahepatic biliary obstruction and lowest in normal liver. These results show that the proliferating biliary epithelium in hepatolithiasis contains amylase isoenzymes and trypsin and that biliary epithelium retains the ability to produce these enzymes after proliferation, suggesting that a large amount of amylase isoenzymes and trypsin may be secreted into the bile ducts in hepatolithiasis. These enzymes may play an important role in the pathophysiology of hepatolithiasis.     

216例恶性阻塞性黄疸患者手术疗效的因素分析

目的 探讨影响恶性阻塞性黄疸临床治疗的预后因素。方法 对１９９０年１月～１９９７年１２月２１６例恶性阻塞性黄疸手术患者的１７种临床指标进行分析。结果 １７３例（８０．１％）手术后１个月存活,发生并发症８１例,发生率３７．５％。台术者胆管炎、胆瘘、出血、肾功能衰竭等并发症均高于姑息术者,差异均有显著性（Ｐ〈０．０１）;１月生存率和术后并发症发生率与ＡＳＡ分级、黄疸持续的时间等有明显关系（Ｐ〈０．０１     

螺旋CT薄层动态增强扫描对肝外胆管癌的诊断价值

目的：探讨肝外胆管癌螺旋CT薄层动态增强扫描的表现及其诊断价值。方法：回顾性分析29例经手术病理证实的肝外胆管癌螺旋CT表现。CT平扫层厚和间隔为7mm。增强采用薄层动态扫描，分别于注射对比剂后25．60s行动脉期、门静脉期扫描，10～15min行延迟期扫描。层厚和间隔为5mm或3mm。结果：29例均显示梗阻处上方胆管不同程度扩张，梗阻处管壁不规则增厚、管腔非对称性狭窄或腔内软组织结节形成；与平扫相比，薄层动态增强扫描使上述征象显示更加明确，尤其是病变强化具有特征，即动脉期强化不明显，门静脉期轻度强化，延迟期强化程度明显增加，因而有助于肝外胆管癌的诊断。结论：螺旋CT薄层动态增强扫描对肝外胆管癌的诊断具有重要价值。     

乳头括约肌小切开联合大气囊扩张在 肝外胆管大结石内镜治疗中的作用

目的 探讨乳头括约肌小切开联合大气囊扩张术在肝外胆管大结石内镜治疗中的安全性及有效性。方法 87例肝外胆管结石直径≥1.0 cm患者采用奇偶数法分为两组，奇数组43例接受乳头括约肌小切开联合大气囊扩张术取石治疗，偶数组44例接受乳头括约肌切开取石治疗，比较两组间结石取净率、取石次数、机械碎石率、结石嵌顿率、操作时间及早期并发症发生情况。结果 观察组4例出现高淀粉酶血症，3例出现轻症急性胰腺炎，并发症发生率为16.3%（7/43）；对照组中，有3例出现高淀粉酶血症，2例出现轻症急性胰腺炎，3例出血，并发症发生率为18.2%（8/44），差异无显著性（P >0.05），但出血率比较差异有显著性（P <0.05）。观察组结石取净率、结石嵌顿率、机械碎石率、二次取石率、操作时间分别为97.7%（42/43）、0%（0/43）、4.6%（2/43）、0%（0/43）及（35.2±5.2）min；对照组对应为97.7%（43/44）、6.8%（3/44）、18.1%（8/44）、13.6%（6/44）及（45.1±4.0）min，除结石取净率比较差异无显著性（P >0.05），其他4项比较差异均有显著性（P <0.05）。结论 乳头括约肌小切开联合大气囊扩张术在肝外胆管大结石内镜治疗中安全有效，操作简便。     

Targeting the PI3K/AKT/mTOR pathway in biliary tract cancers: A review of current evidences and future perspectives

Biliary tract cancers (BTCs) are a group of invasive neoplasms, with increasing incidence and dismal prognosis. In advanced disease, the standard of care is represented by first-line chemotherapy with cisplatin and gemcitabine. In subsequent lines, no clear recommendations are currently available, highlighting the need for novel therapeutic approaches.The PI3K/AKT/mTOR pathway is a core regulator of cell metabolism, growth and survival, and is involved in BTCs carcinogenesis and progression. Mutations, gene copy number alterations and aberrant protein phosphorylation of PI3K, AKT, mTOR and PTEN have been thoroughly described in BTCs and correlate with poor survival outcomes.Several pre-clinical evidences state the efficacy of PI3K/AKT/mTOR pathway inhibitors in BTCs, both in vitro and in vivo. In the clinical setting, initial studies with rapamycin analogs have shown interesting activity with an acceptable toxicity profile. Novel strategies evaluating AKT and PI3K inhibitors have risen serious safety concerns, pointing out the need for improved patient selection and increased target specificity for the clinical development of these agents, both alone and in combination with chemotherapy.This review extensively describes the role of the PI3K/AKT/mTOR pathway in BTCs and examines the rationale of its targeting in these tumors, with particular focus on clinical activity, toxicities and perspectives on further development of PI3K/AKT/mTOR pathway inhibitors.     

Repeated resection for extrahepatic recurrences after hepatectomy for ruptured hepatocellular carcinoma

In March 1999, a 54-year-old man with chronic hepatitis C was referred to our hospital because of ruptured hepatocellular carcinoma (HCC) located in Couinaud’s segments 4 and 8. He underwent central bisegmentectomy of the liver with partial resection of the diaphragm. After the first surgery, extrahepatic metastases were found on different occasions in the abdominal wall, thoracic cavity, and greater omentum and were all surgically resected. In February 2001, the serum protein induced by vitamin K absence or antagonist-II (PIVKA-II) level increased markedly to 19?000?mAU/l. Magnetic resonance imaging showed a massive right subphrenic tumor with invasion to the right diaphragm and posterior segment of the liver. The patient underwent en bloc resection of the tumor, diaphragm, posterior segment of the liver, and right lower pulmonary lobe. After the surgery, the PIVKA-II level rapidly decreased, and it has remained within the normal range to date. Two years after the last surgery, the patient is doing well without any extrahepatic recurrence, although small intrahepatic recurrences have been completely treated by radiofrequency ablation and transcatheter arterial chemoembolization. Ruptured HCC often exacerbates the risk of peritoneal dissemination and is usually difficult to completely resect. This is an extremely rare case of a patient who successfully underwent five repeated resections for extrahepatic recurrences after hepatectomy for ruptured HCC.