肝内胆管囊腺瘤与囊腺癌的诊治体会

目的 探讨肝内胆管囊腺瘤与囊腺癌的诊断与治疗.方法回顾性分析本院2004年1月至2013年10月收治的5例肝内胆管囊腺瘤、囊腺癌临床资料.结果 5例均行手术治疗,其中1例行右半肝切除术,3例行不规则肝切除术,5例均痊愈出院.术后1例失访,4例随访,其中1例囊腺癌术后11个月死于肿瘤复发转移,3例囊腺瘤现存活.结论肝内胆管囊腺瘤和囊腺癌的治疗以外科手术切除为主,加强对肝内胆管囊腺瘤与囊腺癌的认识,提高疾病诊断率对于指导治疗和改善预后至关重要.     

原发性肝内胆管囊腺癌（附5例报告）

目的 探讨原发性肝内胆管囊腺癌的诊断及外科处理.方法 回顾1982年1日至1998年底收治的5例原发性肝内胆管囊腺癌诊治经验并结合文献贸料加以讨论.结果 5例中2例行根治性手术切除,分别存活了4.5年和3年,另3例肿块不能根治性切除,2例存活8目和20个月,1例已治疗5月,仍在治疗中.结论 原发性肝内胆管腺癌诊断主要依靠影像学和经皮肝穿刺活检,最佳的治疗方法是肿块的根治性切除.如果肿块能完整切除,预后较好.     

肝内胆管囊腺癌17例临床分析

目的 分析肝内胆管囊腺癌的临床特点及治疗,探讨影响预后的相关因素.方法 回顾性分析2002年1月至2007年9月共17例经手术病理证实为肝内胆管囊腺癌的临床资料,并进行随访.对预后的影响采用Cox回归分析和Kaplan-Meier分析.结果 17例中男4例,女13例,平均年龄为49.3岁.患者以上腹痛为主要临床表现,仅2例患者CA19-9异常,2例CEA异常,17例患者AFP均正常.17例中行根治性手术7例,肿瘤完全切除联合取瘤栓术7例,姑息性手术3例.病理提示胆管囊腺癌10例,胆管囊腺瘤局部癌变2例,乳头状胆管囊腺癌2例,黏液性乳头状胆管囊腺癌2例;1例为肝内胆管囊腺癌与肝细胞癌双结节混合性癌.17例中肿瘤高分化7例,中分化3例,低分化7例.手术方式(β=-0.692,P=0.01)、肿瘤分化程度(β=-2.041,P=0.007)对预后影响有统计学意义.结论 肝内胆管囊腺癌以成年女性多见.血清CA19-9对诊断无帮助.肝内胆管囊腺癌的胆总管癌栓并不预示临床预后差,行根治性手术或根治性切除联合取栓术均能有较好的预后.     

肝胆管囊腺瘤9例诊治体会并文献复习

目的探讨肝胆管囊腺瘤的诊断及治疗方法 ,从而提高对本病的认识。方法总结我院2004年1月至2015年2月收治的9例病理诊断为肝内胆管囊腺瘤的临床表现、影像学特点、外科手术治疗情况及术后病理结果,回顾性分析各诊断及治疗情况。结果 7例肝脏囊腺瘤患者均为女性,年龄分布在42~76岁之间,平均62岁,影像学肿瘤病灶7例位于肝左叶,为囊实性。9例病变均施行手术完整切除,术后病理诊断为肝内胆管囊腺瘤。5例随访2年,未见复发、恶变。结论肝脏囊腺瘤多好发于中年女性,临床表现不典型,术前易误诊,PTC胆道造影结合CT及MRI检查有助于明确诊断,治疗上首选手术治疗,准确病变定位是手术切除的关键,推荐病变肝叶或肝段的完整切除。     

肝内胆管囊腺瘤17例诊治分析

目的 探讨肝内胆管囊腺瘤的诊断和治疗。 方法 回顾性分析2003年1月至2009年12月中国医科大学附属盛京医院收治的17例肝内胆管囊腺瘤病人的临床资料。 结果 主要临床表现为上腹部不适或疼痛。2例曾误诊为肝囊肿行开窗引流术。6例病人CA19-9升高,1例甲胎蛋白（AFP）轻度升高,癌胚抗原（CEA）水平均正常。7例术前影像学检查确诊。均行根治性肝切除手术。病理结果显示肝内胆管囊腺瘤14例,胆管囊腺瘤恶变3例。术后随访8~60个月无复发。 结论 肝内胆管囊腺瘤术前难以确诊,易恶变,根治性肝切除手术疗效满意。     

肝内胆管囊腺瘤与囊腺癌的诊断及外科治疗

目的 探讨肝内胆管囊腺瘤和囊腺癌的诊断和治疗方法.方法 对中国医学科学院肿瘤医院1996年1月至2007年10月收治的8例肝内胆管囊腺瘤和囊腺癌的临床资料进行回顾性分析.结果 肝内胆管囊腺瘤和囊腺癌无特征性临床表现.术前AFP均为阴性;B超和CT的诊断正确率分别为75%(6/8)、87.5%(7/8).8例病人均行手术切除,7例行肝肿瘤不规则切除术,1例行左半肝切除术.术后病人恢复良好.8例均获得随访,除1例囊腺癌术后3年死于肿瘤复发转移外,其余均生存良好.结论 肝内胆管囊腺瘤与囊腺癌主要依靠影像学和病理诊断,手术切除可以获得满意的疗效.     

肝内胆管囊腺瘤诊治分析(附12例报道)

目的探讨肝内胆管囊腺瘤的临床表现、诊断和治疗方法。方法回顾性分析2004年1月至2013年12月期间笔者所在医院诊治的12例肝内胆管囊腺瘤患者的临床资料。结果女10例,男2例;平均年龄49.0岁(16~77岁);病程为1个月~3年,平均病程7个月。临床表现为右上腹饱胀感伴隐痛6例、右上腹肿块1例、黄疸1例,无症状体检发现4例。增强CT检查示肿瘤包膜完整、内有分隔者11例,分隔之间密度不一致;增强后内容物无强化,但囊壁及分隔可见轻度强化者9例。12例均行手术治疗,并完整切除肿瘤。仅有1例腺瘤恶变者于术后2年复发,另11例均无复发。结论肝内胆管囊腺瘤好发于中年女性,主要临床症状为上腹饱胀感伴隐痛、右上腹肿块、黄疸等;增强CT是其主要的术前诊断方法。手术完整切除肿物是最佳的治疗方案,可以有效防止其复发。     

肝内胆管囊腺癌7例诊治分析

目的总结肝内胆管囊腺癌的诊断及治疗方法。方法回顾性分析笔者所在医院2000年4月至2013年7月期间收治的7例肝内胆管囊腺癌患者的临床资料。结果 7例患者均经实验室检查和影像学检查初步诊断为肝脏占位,但确诊依据的是术后病理学检查。5例行根治性手术切除,其中4例行半肝切除术(非浸润型),随访期间均存活,1例行囊壁完整切除术(浸润型)者于术后18个月死亡;1例行囊壁大部分切除术(浸润型),于术后12个月死亡;1例行彩超引导下的肝脏穿刺囊内积液引流、细针穿刺活检术(浸润型),于术后7个月死亡。结论肝内胆管囊腺癌的影像学表现多为肝脏囊性病变,但确诊仍需病理学检查。肝内胆管囊腺癌局限于囊内者,通过行完整切除即可获得较满意的临床疗效;而癌细胞向囊壁外肝组织浸润生长者的预后差,生存时间短。     

误诊为肝包虫病的肝内胆管囊腺瘤一例

肝内胆管囊腺瘤是一种比较罕见的肝内囊性病变,具有一定的恶变倾向及较高的复发率。该病多发于中年女性,早期不易察觉,缺少特异性临床表现及实验室检查结果,易造成误诊。本文报道了1例误诊为肝包虫病的肝内胆管囊腺瘤病例。患者女性,38岁,有牧区生活史,影像学检查提示肝包虫病。患者行肿物完整切除术,术中发现肿物囊泡不同于传统肝包虫囊泡,术后病理诊断为肝内胆管囊腺瘤。     

肝内胆管囊腺瘤和囊腺癌的CT、MRI及病理表现

目的探讨肝内胆管囊腺瘤和囊腺癌的CT、MRI和病理特点。方法回顾性分析经手术病理证实的6例肝内胆管囊腺瘤和2例肝内胆管囊腺癌的影像及临床病理资料，将病变的影像表现与其病理大体形态及组织学表现作对照分析。结果6例肝内胆管囊腺瘤，女4例、男2例；2例肝内胆管囊腺癌均为女性病人；8例病人平均年龄55岁。所有病灶均表现为多房囊性肿块，肿瘤囊腔各分房内常为多种液体成分，在CT上可表现为不同密度、在MRI上可表现为不同信号强度。囊内出现多发大小不等的壁结节在胆管囊腺癌内更常见，囊内有分隔但无壁结节只见于胆管囊腺瘤。在7例CT扫描中，4例胆管囊腺瘤和1例胆管囊腺癌可见囊壁或分隔上钙化，囊壁、囊内分隔及囊内结节均为轻、中度延迟增强。肿瘤中出现卵巢样间质见于3例胆管囊腺瘤和1例胆管囊腺癌，且均为女性病人。结论肝内胆管囊腺瘤和囊腺癌是肝脏不常见的囊性肿瘤，影像上多房、囊内有分隔且各分房囊内密度或信号不一致，高度提示肝内胆管囊腺瘤或囊腺癌的诊断，如囊内伴有多发大小不等的结节，则进一步提示囊腺癌的可能。但影像学表现不能区分肿瘤中有无卵巢样间质。     

Anatomic and molecular pathology of intrahepatic cholangiocarcinoma

Intrahepatic cholangiocarcinoma (ICC) is the second most common malignant tumor of the liver, and ICC is reportedly increasing recently. ICC is usually adenocarcinoma with variable desmoplastic reaction, although there are several special or unusual histological features. ICC may arise at the large intrahepatic bile duct near the hepatic hilus and also from the bile ductules at the border of the hepatic parenchyma. On the anatomical level, the pathology of ICC differs depending on the region from which the ICC arises. At the large intrahepatic bile duct, ICC presents papillary growth and periductal infiltration. Some cases show extensive papillary growth and intraluminal spread with marked gastroenteric metaplasia. Mucus core protein 1 is expressed in aggressive ICC. ICC arising from ductules shares phenotypes of hepatocellular carcinoma. ICC in chronic biliary diseases, particularly arising in hepatolithiasis, presents precancerous lesions that include biliary epithelial dysplasia, as well as in-situ carcinoma. Chronic advanced hepatitis C is one of the background diseases of ICC. Chronic inflammation, with the upregulation of cyclooxygenase-2 and growth factors, and the formation of reactive oxygen species are one of the causative factors in the DNA damage of biliary epithelial cells. K-ras mutation and aberrant expression of p53 are found in one-third of ICCs. The latter may be due to mdm-2 upregulation. Hepatocyte growth factor/met and interleukin 6 (IL6)/IL6 receptor are involved in cell proliferation/mitoinhibition and apoptosis in ICC. Fibrous stroma formation and invasion involve the proliferation of -smooth muscle antigen-positive stromal cells, and cell-to-cell and cell-to-matrix interactions involving E-cadherin/catenin and CD44 and matrix proteinases may be involved in the invasion of ICC. Evasion of immune surveillance involving the Fas/FasL system is important in the malignant progression of ICC. Further molecular and genetic studies are mandatory to evaluate the pathogenesis and progression of ICC.     

重症急性梗阻性化脓性肝胆管炎的临床特点

目的　提高对重症急性梗阻性化脓性肝胆管炎 (单纯肝内胆管梗阻的重症胆管炎称肝内型ACST)的诊断治疗水平 ,减少其死亡率。方法　回顾 1991～ 2 0 0 0年本院收治的 186例重症胆管炎 (ACST)中 2 8例肝内型ACST的临床资料 ,对其临床表现和治疗进行分析探讨。结果　 2 8例肝内型ACST出现明显腹痛 8例 ,黄疸 12例 ;15 8例胆总管型ACST出现Charcot三联症 14 9例。将两组的腹痛和黄疸发生率行 χ2 检验 ,两组差异有显著性 (P <0 0 1)。结论　肝内胆管梗阻的重症胆管炎临床表现和治疗有一定的特殊性 ,早期诊断、及时手术能减少其死亡率。     

Relief of intractable pruritis in Alagille syndrome by partial external biliary diversion

Background

Patients with Alagille syndrome (AGS) may develop pruritis, skin hypertrophy, and xanthomas because of chronic cholestasis and hypercholesterolemia. Partial external biliary diversion (PEBD) has been used successfully to treat chronic cholestasis in patients with progressive familial intrahepatic cholestasis (PFIC) and is a potentially useful treatment for patients with severe and intractable pruritis because of AGS.

Methods

Four children with chronic cholestasis and intractable pruritis were treated with PEBD, 1 by surgical cholecystostomy and 3 by cholecystojejunostomy.

Results

Three patients had a known diagnosis of AGS. The fourth was an 11-month-old infant boy with PFIC. The first patient, a 15-month-old boy with AGS, underwent surgical cholecystostomy, which has required frequent tube changes to maintain patency. Three patients underwent PEBD using a segment of jejunum as a conduit between the gallbladder and the skin, where bile is collected in a standard ostomy appliance. Mean follow-up is 15.5 months (range, 9-26 months).All patients experienced rapid and enduring relief of pruritis. Two adolescents with AGS had significant improvement of the hypertrophic skin of their hands. There was one significant complication: the infant with PFIC required reoperation for bleeding from the jejunal anastomosis 1 week after PEBD; he has subsequently done well. One adolescent girl with AGS initially had difficulty with her ostomy because of poor site placement and partial retraction, but nevertheless has managed quite well. The patient treated by cholecystostomy has had excellent relief of his pruritis and is being considered for conversion to cholecystojejunostomy.

Conclusions

Chronic cholestasis caused by AGS can cause debilitating symptoms that are resistant to medical therapy. Partial external biliary diversion is a safe and technically straightforward operation that may be effective for the relief of intractable pruritis and other symptoms in patients with AGS.     

肝胆管结石在胆道外科中的重要地位

回顾性调查７１８４例胆石症患者的临床资料，分析了胆石部位与性别、年龄、职业的关系，胆石部位在不同医院的分布以及胆石部位与病死率的关系。认为在城市居民和城市综合医院里，胆囊结石占胆石症的比例呈明显上升趋势，但同样的在相当长的一段时期里，在农村居民和地县基层     

Intrahepatic biliary cysts in children with biliary atresia who have had a Kasai operation

Purpose

In patients with biliary atresia who had undergone a Kasai operation, treatment of intrahepatic biliary cysts (IBCs), particularly when complicated by cholangitis, is often difficult because the clinical implications and the course of IBCs are unclear. Thus, to determine the best treatment guideline, the morphology of IBCs, the clinical course, and the outcomes of such patients were evaluated.

Patients and Methods

A total of 44 patients with type III biliary atresia who underwent a Kasai operation from 1977 to 2005 were postoperatively examined for IBC by using ultrasonography and computed tomography. We classified the IBCs based on their number and shape.

Results

Intrahepatic biliary cysts developed in 12 of 54 patients. Three patients with solitary simple cysts and 1 patient with multiple simple cysts had no history of cholangitis. Two patients with multiple simple cysts had cholangitis at the time of IBC diagnosis and were treated with percutaneous transhepatic cholangiodrainage (PTCD). Patients with simple IBCs did not develop persistent cholangitis and their prognosis depended largely on their liver function; 3 of 6 patients remained healthy without cholangitis, whereas 3 patients required liver transplantation (LT) because of progressive liver failure or worsening hepatopulmonary syndrome, and not severe cholangitis. On the other hand, all 6 patients with multiple complicated IBCs had persistent cholangitis, eventually requiring LT. Even after bile flow to the intestine was reestablished after PTCD, both IBCs and cholangitis recurred. These patients required LT because of severe cholangitis.

Conclusions

Intrahepatic biliary cysts without cholangitis are not a source of infection and require no treatment. Simple IBCs with cholangitis can be controlled by antibiotics and/or PTCD. Patients with multiple complicated IBCs have a poor prognosis, requiring LT to control cholangitis. Although PTCD can control cholangitis in these patients as they wait for LT, PTCD does not alleviate it—LT is the final solution.     

冷保存对肝移植术后肝内胆管微循环的影响

目的探讨供肝冷保存对肝移植术后肝内胆管微循环的影响。方法实验大鼠随机分为假手术组（SO组）、供肝冷保存1h组（CP1h组）、供肝冷保存24h组（CP24h组）。采用重建肝动脉的大鼠肝移植模型。在肝移植术后的不同时间点,观察肝内胆管组织损伤程度;经肝动脉注入微球,光镜下行肝组织汇管区内微球计数;采用间接免疫荧光双染技术检测肝组织汇管区微小血管内皮细胞eNOS、El＂-1和ICAM-1的表达,并采用原位杂交技术检测其mRNA的表达。结果冷保存再灌注可引起肝内胆管结构改变,冷保存时间越长损害程度越重。冷保存再灌注可引起肝组织汇管区内微球数量增加,且时间越长微球数量增加越明显。冷保存再灌注可引起汇管区微小血管内皮细胞eNOS蛋白及mRNA表达水平降低,而ET-1和ICAM-1的蛋白及mRNA表达水平升高。结论冷保存可引起大鼠移植肝脏肝内胆管微循环及其内皮细胞功能明显改变,微循环障碍可能在肝内胆管冷保存再灌注损伤中起重要作用。     

肝内胆管囊腺瘤再次手术临床分析

目的探讨肝内胆管囊腺瘤再次手术的诊治特点。方法回顾性分析总结我院4例肝内胆管囊腺瘤根治性切除术后再次手术的诊治特点。结果肝内胆管囊腺瘤再次手术2例腹胀2例无明显症状,B超、CT、MRI可发现肝内占位难以确诊,再次手术均行根治性切除术,术后病理提示:囊腺瘤复发1例,囊腺癌1例,原发性肝癌1例,肝恶性纤维细胞瘤1例,术后均无明显并发症,随访2例死亡,生存时间为7个月与21个月,1例囊腺瘤痊愈至今未复发,1例肝内胆管囊腺癌随访18个月健在。结论肝内胆管囊腺瘤术后再发肝占位性质难以术前确诊,首选手术治疗,严格术前评估能够获得较好的治疗效果。     

Resection of metastatic liver cancer in a patient with a left-sided gallbladder and intrahepatic portal vein and bile duct anomalies: A case report

INTRODUCTIONThe presence of left-sided gallbladder is closely associated with multiple combined anomalies of the portal vein, hepatic vein, hepatic artery, and bile duct. This requires special attention for preoperative evaluation for the purpose of preventing postoperative complications.PRESENTATION OF CASEA 70-year-old woman with metastatic liver cancer and intrahepatic portal vein, biliary system and hepatic artery anomalies with left-sided gallbladder is reported. On computed tomography (CT), a solitary low density mass occupied from the right anterior to the posterior segment of the liver. The gallbladder bed was on the left of the hepatic fissure. On drip-infusion-cholangiography (DIC) CT three-dimensional (3D) reconstruction, the left medial bile duct arose from the right umbilical portion after arising from the left lateral bile duct. Following a right hepatectomy and lymph node dissection of the hepatoduodenal ligament, hepaticojejunostomy was conducted separately to the left medial and left lateral bile duct.DISCUSSIONThe left-sided gallbladder accompanies with several anomalies of hepatic vascular and bile duct anomalies in a frequent manner. A safe hepatectomy needs accurate operative plans to ascertain the range of hepatectomy, because it often has the diversity of a combined anomaly.CONCLUSIONPreoperative DIC-CT 3D reconstruction was extremely useful because it provided an important information that could not be obtained with 2D-DIC-CT. 3D imaging has the ability to demonstrate complex anatomical relationships, this devise is a effective new tool for making appropriate preoperative strategy.     

手术结合纤维胆道镜治疗肝内胆管狭窄及肝内胆管结石的疗效分析

目的探讨手术结合纤维胆道镜治疗肝内胆管狭窄及肝内结石的疗效。方法通过T管窦道胆道镜球囊扩张治疗肝内胆管狭窄、取净肝内结石。结果81例肝内胆管结石患者合并肝内胆管狭窄43例，占53．09％；利用胆道球囊反复扩张，解除狭窄的成功率88．37％；取净结石的成功率100％。术后所有病例无严重并发症。结论纤维胆道镜治疗肝内胆管狭窄克服了外科手术的盲区，具有创伤小，治愈率高，并发症少，疗效确切的优点。内镜与手术的结合使肝内胆管狭窄的治疗取得了十分满意的疗效。     

How can cholangioscopy improve the management of intrahepatic stones?

Summary Experience with endoscopic treatment of intrahepatic stones in 93 cases encountered over 13 years starting in 1974 was analyzed and the usefulness of the procedure is discussed in this paper. Postoperative cholangioscopy are percutaneous transhepatic cholangioscopy are easy, safe and reliable procedures because they can be carried out under both fluoroscopic and direct visual control. They can be safely and repeatedly carried out if the sinus tract is kept open. Preoperative percutaneous transhepatic cholangioscopy is an important tool in the diagnosis and treatment of intrahepatic stones, because it provides the important information necessary for selection of an appropriate therapeutic procedure. Different stone extraction techniques can be accurately carried out with endoscopic guidance, and stones that are visible are readily removable. A good prognosis can be expected when stones are completely extracted. With this approach, the author believes that the results of treatment for intrahepatic stones will be significantly improved. Presented at the International Congress on Surgical Endoscopy, Ultrasound, and Interventional Techniques, Berlin 1988