1例IgG4相关性硬化性胆管炎误诊为胆管癌的病例分析并文献复习

目的探讨IgG4相关性硬化性胆管炎的临床表现、诊断、鉴别诊断及治疗。方法对2014年在四川大学华西医院就诊的1例误诊为胆管癌的IgG4相关性硬化性胆管炎患者的临床资料进行回顾性分析,包括既往误诊为胆管癌的病史、实验室及辅助检查等,结合相关文献分析误诊的原因并总结经验,以指导日后的临床工作。结果该病例术前高度怀疑为肿瘤,行手术治疗后病理报告未查见确切癌细胞,后因黄疸多次安置、更换胆管支架,最终诊断为IgG4相关性硬化性胆管炎,予以激素治疗后好转。结论 IgG4相关性硬化性胆管炎是较为少见的胆管系统疾病,其诊断较困难,与胆管癌的鉴别十分重要。     

IgG4硬化性胆管炎误诊为肝门胆管癌及术后消化道出血MDT讨论

目的探讨免疫球蛋白G4硬化性胆管炎(IgG4SC)误诊为肝门胆管癌以及术后胃十二指肠假性动脉瘤破裂的临床表现、诊断和治疗措施,以提高对该病的认识及合理诊治。方法回顾性分析四川大学华西医院诊治的1例IgG4SC误诊为肝门胆管癌且术后消化道出血患者的临床资料。结果该例患者以肝门胆管癌收入院,术前影像学检查表现为典型的肝门胆管癌影像学特征,遂行肝门胆管癌根治术,术后病理结果诊断为IgG4SC,补查血清IgG4仅轻度升高。术后1个月突发呕血,动脉造影显示胃十二指肠假性动脉瘤破裂出血,经栓塞治疗后痊愈。结论术前诊断IgG4SC较为困难,特别应与肝门胆管癌鉴别,可避免不必要的手术。胃十二指肠假性动脉瘤形成的原因主要是胃十二指肠动脉术中被损伤,肝胆疾病手术行动脉骨骼化过程中需注意动脉保护。临床怀疑假性动脉瘤形成时,应尽早行血管介入诊断并同时行栓塞治疗。     

IgG4相关硬化性胆管炎5例诊治分析

回顾性分析湖南省人民医院收治的5例IgG4相关硬化性胆管炎行手术治疗患者的临床和随访资料,从临床及影像学角度分析其临床诊治特点。术前1例诊断为肝门胆管癌Ⅳ型,4例诊断为胆总管下段癌;3例行胰十二指肠切除术,2例行胆肠内引流术;术后病例均诊断为IgG4相关硬化性胆管炎。笔者认为IgG4相关硬化性胆管炎缺乏典型的临床表现、...     

原发性肝内胆管结石合并胆管癌

目的 总结原发性肝内胆管结石合并胆管癌的临床特征、诊断方法、治疗手段及预后.方法 回顾性分析北京大学第三医院1958年6月至2011年3月收治的原发性肝内胆管结石合并胆管癌709例的临床资料.结果 709例原发性肝内胆管结石合并胆管癌20例(2.8％),获得随访17例,随访率为85％,中位随访时间2(0 ～15)年.胆管癌诊断前原发性肝内胆管结石病程15±11年(3 ～38年).有胆管炎的14例,其中重症胆管炎11例.合并肝脓肿12例,肝硬化或门静脉高压症15例.术前辅助检查提示合并胆管癌诊断的依次为CT、MRCP、B超、肿瘤标志物等.胆管癌的术前临床诊断率为55％ (11/20).行胆管癌根治术4例,姑息性手术7例,探查活检术6例,非手术治疗3例.根治术后1例失访,其余3例均存活,目前存活时间分别为1年1例,5年2例.姑息性手术7例,术后失访2例,存活时间1年2例,3年1例,5年1例.未切除者存活时间均未超过1年.结论 对于原发性肝内胆管结石病例有以下情况者应提高警惕,(1)病史较长、有胆管炎.(2)合并肝硬化.(3)影像学提示胆管壁增厚或肝内占位病变.(4)肿瘤标志物升高.这些提示发生胆管癌的可能性大.对这些病例应严格定期随访,以便早期发现,获得根治切除者预后较好.     

IgG4相关硬化性胆管炎5例诊治分析

回顾性分析湖南省人民医院收治的5例IgG4相关硬化性胆管炎行手术治疗患者的临床和随访资料, 从临床及影像学角度分析其临床诊治特点。术前1例诊断为肝门胆管癌Ⅳ型, 4例诊断为胆总管下段癌;3例行胰十二指肠切除术, 2例行胆肠内引流术;术后病例均诊断为IgG4相关硬化性胆管炎。笔者认为IgG4相关硬化性胆管炎缺乏典型的临床表现、无特殊生化检查以及影像学结果, 常常误诊为其他疾病, 明确诊断通常依赖于临床、影像学、组织学、血清学、治疗及预后的综合分析。     

胆管乳头状瘤14例的临床病理及外科治疗

目的 探讨胆管乳头状瘤的临床病理特点、诊断及治疗方法。方法 对1993年1月至2006年6月经病理证实的14例胆管乳头状瘤的临床资料进行回顾性分析。结果 胆管乳头状瘤的主要临床表现为上腹部疼痛、黄疸及急性胆管炎。胆管乳头状瘤单发6例，多发8例；肿瘤位于肝外胆管6例，肝内胆管5例，肝内外胆管3例；伴轻中度不典型增生5例，癌变6例。术前影像学确诊2例。术后胆道镜检查发现病灶2例。14例患者均行手术治疗，6例肿瘤早期完全切除者术后无复发，3例癌变浸润者生存期在8个月内。结论 胆管乳头状瘤是具有很高恶变潜能的癌前疾病，术前确诊困难。MRCP和胆道镜检查有较高的诊断价值。胆管乳头状瘤单发者应早期行肿瘤局部切除，多发者治疗困难；根治性手术切除是主要治疗方法。     

肝门胆管癌的MDCT诊断及其术前评价

目的 探讨MDCT在肝门胆管癌术前评估中的价值.方法 回顾经病理证实的肝门胆管癌31例,分析术前MDCT检查,观察肿瘤病灶大小、部位、强化方式、周围及远处侵犯情况、肝内胆管扩张以及肝脏、胆囊等改变,并与手术及术后病理结果对照.结果 该组中MDCT定性诊断符合率达93.5%.77.4%的病例MDCT判断梗阻部位及Bismuth-Corlette分型与手术结果相符.MDCT判断分期与临床及TNM分期总的符合率为71.0%,Ⅰ期达100%.结论 MDCT能够较好地检出肝门胆管癌和评价侵犯范围.有助于术前诊断及治疗方案的制定.     

肝门部胆管癌高位切除并回盲部间置-肝门回肠吻合术2例

目的：探索肝门部胆管癌高位切除并回盲部间置－肝门回肠吻合术的临床应用。方法：对2例肝门部胆管癌患者进行详细的术前评估和充分的术前准备,行肝门部胆管癌高位切除并回盲部间置－肝门回肠吻合术,术后给予预防感染、营养支持等治疗,随访3个月,总结临床资料。结果：2例患者术后病理证实为肝门部胆管癌Bismuth IV型,术中均未输血,术后胆管支架管造影示回肠肠袢及肝内无明显气体。钡餐透视提示钡剂进入盲肠,未反流入回肠袢;口服钡剂后9h透视提示盲肠肠袢和吻合部位空肠内钡剂排出,盲肠袋黏膜皱襞有少量钡剂残留,钡剂通过回肠一结肠吻合口时无延迟及明显过快,无明显钡剂反流。2例患者随访3个月,无反流性胆管炎发生。结论：肝门部胆管癌行高位切除、回盲部间置、肝门回肠吻合术是可行的。     

肝细胞癌合并胆管癌栓二例并文献复习

华中科技大学同济医学院附属武汉中心医院2021年收治肝细胞癌合并胆管癌栓2例,其中1例由于肝内未见明显病灶且甲胎蛋白(AFP)阴性,术前极易误诊为胆管癌,另1例为高龄病人肝癌术后复发合并胆管癌栓,经保守治疗之后病情好转。结合该2例病例,作者对肝细胞癌合并胆管癌栓的临床诊治进展进行文献复习,分析该病的发病机制、诊断及治疗方式等。结论：肝细胞癌侵犯肝内外胆管形成胆管癌栓在临床上较少见,易被误诊为胆管癌、胆道结石、胆管炎等,目前其术前诊断主要依赖于影像学检查,治疗上采用手术治疗为主的综合治疗。     

肝胆管结石合并胆管癌的临床特点及防治

目的 探讨肝内胆管结石合并肝内胆管癌的临床特点及其防治.方法 对1990-2009年肝内胆管结石伴肝内胆管癌84例的临床资料进行回顾性研究.结果 肝内胆管结石合并肝内胆管癌的发生率占同期肝内胆管结石病例的4.6%(84/1840),术前明确诊断47例;肿瘤均发生于含结石的胆管处,以左肝多见;病程1～40年,平均18年.20例迟发性肝内胆管癌发生于取石后6-16年,平均9年.临床表现为久治不愈的肝脓肿、难以控制的肝内感染、肝内阻塞性进行性黄疸和影像学提示结石部位的肿瘤性改变.84例中晚期病例65例(65/84,77.4%).行根治性切除者仅35例,姑息性切除26例,射频消融4例,单纯活检19例.结论 (1)肝内胆管结石并发肝内胆管癌的概率较高.(2)对所有肝内占位性病变行术前、术中活检是避免误漏诊的重要方法.(3)早期诊断者行根治性切除可获得良好疗效.(4)对肝内结石伴胆管狭窄、肝段萎缩纤维化者行病灶肝段切除对继发胆管癌有预防作用.     

Usefulness of Steroid Administration for Diagnosis of IgG4-Related Sclerosing Cholangitis

Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is one of the IgG4-related systemic sclerosing diseases and responds well to steroid therapy. A 58-year-old male was admitted with hilar bile duct stenosis revealed by computed tomography. We performed percutaneous transhepatic right portal vein embolization (PTPE) and scheduled a right hepatectomy because a hilar cholangiocarcinoma was first suspected. However, there was no cytologic evidence of malignancy and serum IgG4 was elevated. Steroid therapy was initiated after PTPE. There was no evidence of bile duct stenosis after 4 weeks. Improving diagnostic technique, IgG4-SC was diagnosed and treated with steroid therapy. In some cases, we couldn''t deny the malignancy and performed unnecessary resection. We recommend that steroid administration while waiting for the liver volume to increase after PTPE is useful. The therapy aids in the diagnosis of bile duct stenosis, which has value for a hilar bile duct limit type of IgG4-SC, as in the case reported here.     

Recognition of other organ involvement might assist in the differential diagnosis of IgG4-associated sclerosing cholangitis without apparent pancreatic involvement: report of two cases

IgG4-associated sclerosing cholangitis (IAC) was recently defined as biliary involvement of IgG4-related systemic disease. It is frequently associated with autoimmune pancreatitis, characterized by pancreatic enlargement and irregular narrowing of the pancreatic duct. However, a few cases of IAC with no apparent pancreatic involvement have been described, the characteristics of which may mimic those of cholangiocarcinoma. We report two rare cases of IgG4-associated sclerosing cholangitis at the hepatic hilum, mimicking hilar cholangiocarcinoma. When trying to establish the diagnosis, we should consider other organs that could be involved, such as the pancreas, salivary glands, retroperitoneum, lymph nodes, and kidneys, as well as chronic inflammatory changes. By recognizing these lesions and measuring serum IgG4, IAC can be diagnosed correctly, thereby avoiding unnecessary major surgery for a condition that is treated effectively by steroid therapy.     

Usefulness of transpapillary cytology and biopsy in diagnosing cholangiocarcinoma that mimics primary sclerosing cholangitis

Two patients with a sclerosing type of cholangiocarcinoma are reported. One, a 68-year-old male, presented with jaundice and mild right upper abdominal pain. Endoscopic retrograde cholangiography showed diffuse narrowing and irregularity of the intra- and extrahepatic bile ducts, suggestive of primary sclerosing cholangitis. The other patient was a 72-year-old female who complained of slight right upper abdominal pain, lassitude, and anorexia. Both ultrasonography and computed axial tomography demonstrated slight dilatation of the anterior branch of the right hepatic duct, but no mass. Endoscopic cholangiography revealed stenosis of that duct and diffuse irregularity of the other intrahepatic ducts, also suggestive of primary sclerosing cholangitis. In both patients, however, transpapillary cytology of bile demonstrated malignant cells, and biopsy from within the bile duct showed cholangiocarcinoma. Both transpapillary cytology and biopsy, especially the latter, are useful procedures for the diagnosis of this unique clinical entity.     

IgG4-related intrahepatic sclerosing cholangitis resulting in sepsis caused by secondary suppurative inflammation: report of a case

We report a case of pre-disseminated intravascular coagulation caused by secondary suppurative inflammation in a patient with immunoglobulin (Ig) G4-related sclerosing cholangitis. The patient was a 78-year-old man in whom a localized stenosis of the intrahepatic bile duct was found without any other bile duct stricture or symptoms. He underwent surgical resection 6 months later for acute severe cholangitis and sepsis caused by bile duct obstruction. The resected specimen contained an abscess and nodulary mass in the liver. Immunohistochemical analysis revealed IgG4-positive plasma cell infiltration, whereby we diagnosed IgG4-related sclerosing cholangitis. As IgG4-related sclerosing cholangitis limited to within the intrahepatic portion is extremely rare, we present this case with a review of the literature.     

肝吸虫性胆管炎误诊为胆管癌病例分析

目的报道3例肝吸虫性胆管炎的诊治经过,总结临床经验教训。方法对3例肝吸虫性胆管炎误诊为胆管癌临床资料进行回顾性总结分析。结果 3例肝吸虫性胆管炎病人均为男性,年龄50～65岁,均有食鱼生史,1例因"上腹部疼痛4天"入院,1例因"反复上腹痛1月余"入院,1例因"反复上腹疼痛半年"入院,3例术前均误诊为胆管癌,行手术探查,手术方式为胆囊切除、胆总管切开探查、胆道镜检查、T管引流术,术中胆管内均可见大量华支睾吸虫虫体,其中1例形成化脓性胆管炎,术中胆道镜探查肝内外胆管未见明显肿物以及狭窄,术后无明显并发症,均痊愈出院。结论对于高度怀疑胆管癌的肝吸虫性胆管炎患者先驱虫治疗后观察胆管系统情况再决定是否手术探查是比较合适的治疗方案。     

经皮经肝胆道造影术对IgG4相关硬化性胆管炎的诊断价值

目的 分析总结经皮经肝胆道造影术（percutaneous transhepatic cholangiography,PTC）对IgG4相关硬化性胆管炎（IgG4-related sclerosing cholangitis,IgG-SC）的诊断价值。方法 回顾性选取3例初诊时应用PTC的IgG4-SC病例,将胆道造影结果与同期的MRCP及肝门部胆管癌的胆道造影结果相比较。结果 与MRCP相比,PTC更灵活、更清楚地显示出IgG4-SC患者胆管狭窄呈长段、均匀、线性、对称的表现,与肝门部胆管癌表现明显不同。结论 PTC显示IgG4-SC的胆管狭窄有明显优势,有助于IgG4-SC的早期诊断和疗效观察。     

Immunoglobulin G4–Related Sclerosing Cholangitis Mimicking Hilar Cholangiocarcinoma Diagnosed With Following Bile Duct Resection: Report of a Case

We report a rare case of immunoglobulin G4 (IgG4)–related sclerosing cholangitis without other organ involvement. A 69-year-old-man was referred for the evaluation of jaundice. Computed tomography revealed thickening of the bile duct wall, compressing the right portal vein. Endoscopic retrograde cholangiopancreatography showed a lesion extending from the proximal confluence of the common bile duct to the left and right hepatic ducts. Intraductal ultrasonography showed a bile duct mass invading the portal vein. Hilar bile duct cancer was initially diagnosed and percutaneous transhepatic portal vein embolization was performed, preceding a planned right hepatectomy. Strictures persisted despite steroid therapy. Therefore, partial resection of the common bile duct following choledochojejunostomy was performed. Histologic examination showed diffuse and severe lymphoplasmacytic infiltration, and abundant plasma cells, which stained positive for anti-IgG4 antibody. The final diagnosis was IgG4 sclerosing cholangitis. Types 3 and 4 IgG4 sclerosing cholangitis remains a challenge to differentiate from cholangiocarcinoma. A histopathologic diagnosis obtained with a less invasive approach avoided unnecessary hepatectomy.Key words: Immunoglobulin G4, Cholangitis, Cholangiocarcinoma, Autoimmune pancreatitis, Hepatectomy, DiagnosisSerum immunoglobulin G4–related sclerosing cholangitis (IgG4-SC) is a type of autoimmune pancreatitis associated with elevated serum IgG4 levels.^1,2 Types 3 and 4 IgG4-SC are difficult to differentiate from bile duct cancer, and hepatectomy has been reported sporadically in such situations.^3,4 We describe a rare case of a patient with IgG4-SC but without pancreatic lesions. Accurate diagnosis was made, without the need for performing a partial hepatectomy.     

Fibroinflammatory Biliary Stricture: A Rare Bile Duct Lesion Masquerading as Cholangiocarcinoma

Introduction  Fibroinflammatory biliary stricture (FIBS) is a rare benign tumor-like process of the extrahepatic bile duct that masquerades as cholangiocarcinoma. Methods  In order to distinguish this unusual entity from cancer, we performed a systematic analysis of 11 patients with FIBS. All patients presented with jaundice; six patients had coexisting autoimmune disease. Preoperative evaluation included computed tomography scan and endoscopic retrograde cholangiopancreatography with benign brush cytology. Surgical treatment included nine bile duct resections with five concurrent liver resections and two incisional biopsies. Light microscopy demonstrated fibrous lesions admixed with chronic inflammation. Results and discussion  Immunohistochemistry demonstrated smooth muscle actin expression in all lesions except one; five tumors exhibited IgG4 positive plasma cells. The lesions were negative for cytokeratin, ALK1, CD21, S100, Ki67, and p53. Six patients received postoperative immunosuppression. At 41 month median follow-up (range 15–58 months), there was no evidence of recurrent FIBS in ten patients, while one was lost to follow-up. Conclusion  FIBS is a rare myofibroblastic lesion with an immunohistochemical profile distinct from other epithelial and stromal neoplasms of the extrahepatic bile duct. A subset of these cases appear to represent IgG4-related sclerosing cholangitis. Because preoperative cytology is not diagnostic of FIBS, surgical resection remains the mainstay of diagnosis and treatment, while immunosuppression may reduce the risk of recurrence. Poster Presentation at Digestive Disease Week, May 2006, Los Angeles CA, USA.     

IgG4-related sclerosing cholangitis with and without hepatic inflammatory pseudotumor, and sclerosing pancreatitis-associated sclerosing cholangitis: do they belong to a spectrum of sclerosing pancreatitis?

Sclerosing cholangitis (SC) is a heterogeneous disease entity. Different etiologies such as choledocholithiasis, biliary tumor, or pericholangitis can manifest as SC. Hepatic inflammatory pseudotumor (IP) is rarely associated with SC (sclerosing cholangitis associated with hepatic inflammatory pseudotumor; SC-hepatic IP), but sclerosing pancreatitis (SP) is not infrequently associated with bile duct lesions (sclerosing pancreatitis-associated sclerosing cholangitis; SP-SC). In this study, we compared the histologic changes of hepatic hilar and extrahepatic bile duct lesions of SC (7 cases), SC-hepatic IP (5 cases), SP-SC (5 cases), and typical primary sclerosing cholangitis (PSC) (5 cases). Histologically, all SP-SC cases showed extensive and dense fibrosis with marked lymphoplasmacytic infiltration, many eosinophils, and obliterative phlebitis. Four cases of SC showed bile duct lesions similar to those of SP-SC, whereas other three cases of SC showed milder lymphoplasmacytic infiltration, scant eosinophilic cell infiltration, and no obliterative phlebitis. All SC-hepatic IP cases showed bile duct lesions identical to those of SP-SC. Immunohistochemically, many IgG4-positive plasma cells were found in the bile duct lesions of all SP-SC cases, 4 SC cases with marked lymphoplasmacytic infiltration, and all SC-hepatic IP cases. By contrast, IgG4-positive plasma cells were scarce or hardly found in the remaining 3 SC cases and all PSC cases. In conclusion, 4 SC cases and all SC-hepatic IP cases showed bile duct lesions identical to those of SP-SC, suggesting that these three conditions may be a single disease entity. Their pathogenesis may be similar or closely related to that of SP, and in that respect they may represent an IgG4-related biliary disease. They may respond to steroid therapy as SP does.     

Late development of bile duct cancer in patients who had biliary-enteric drainage for benign disease: a follow-up study of more than 1,000 patients

OBJECTIVE: To evaluate the correlation between biliary-enteric surgical drainage and the late development of cholangiocarcinoma of the biliary tract. SUMMARY BACKGROUND DATA: In patients with biliary-enteric drainage, reflux of intestinal contents into the bile duct may occur and cause cholangitis, which is regarded as the most serious complication of these procedures. Lithiasis of the biliary tract and a previous biliary-enteric anastomosis have both been suggested to favor the late onset of cholangiocarcinoma. METHODS: Consecutive patients (n = 1,003) undergoing three different procedures of biliary-enteric anastomosis (transduodenal sphincteroplasty, choledochoduodenostomy, and hepaticojejunostomy) between 1967 and 1997 were included in this study. The postoperative clinical course and long-term outcome were evaluated by a retrospective review of the hospital records and follow-up. Mean follow-up was 129.6 months. RESULTS: Fifty-five (5.5%) cases of primary bile duct cancer were found among the 1,003 patients at intervals of 132 to 218 months from biliary-enteric anastomosis. The incidence of cholangiocarcinoma in the three groups was 5.8% in transduodenal sphincteroplasty patients, 7.6% in choledochoduodenostomy patients, and 1.9% in hepaticojejunostomy patients. The incidence of malignancy related to the different underlying diagnosis was 5.9%, 7.2%, and 1.9% in patients with choledocholithiasis, sphincter of Oddi stenosis, and postoperative benign stricture, respectively. Although only one patient who developed cholangiocarcinoma had previous concurrent lithiasis of the biliary tract, 40 patients had experienced mostly severe, recurrent cholangitis. No case of malignancy occurred in patients scored as having no cholangitis in the early and long-term postoperative outcome. Univariate and multivariate analyses have shown the presence of cholangitis as the only factor affecting the incidence of cholangiocarcinoma. CONCLUSIONS: Chronic inflammatory changes consequent to biliary-enteric drainage should be closely monitored for the late development of biliary tract malignancies.