胆管内乳头状肿瘤临床病理学分析及预后因素评价

目的 探讨胆管内乳头状肿瘤(IPNB)的临床病理学特征及影响恶性病人生存的危险因素。方法 回顾性分析2014年1月至2020年12月浙江大学医学院附属邵逸夫医院收治的33例IPNB病人的临床病理学特征及术后随访资料。比较不同组织学分级及不同解剖分型病人的临床病理学特征,并分析影响恶性IPNB病人生存的危险因素。结果 与良性组相比,恶性IPNB病人年龄较大(P=0.050),CA19-9的中位数水平更高(P=0.004),术前CT或MR检查提示胆管内肿块比例更高(P=0.027),手术切除范围差异有统计学意义(P=0.008)。恶性病人的总生存率低于良性组(P=0.012)。肝内型、肝外型与弥漫型IPNB病人在术前腹痛症状(P=0.013)方面差异有统计学意义。多因素Cox回归分析结果显示,术后复发是恶性IPNB病人不良预后的独立危险因素(P=0.013)。结论改良解剖学分型能够一定程度上指导临床制定手术切除范围,恶性IPNB病人预后相对较差,术后复发与恶性IPNB病人的不良预后显著相关。     

胆管癌相关肿瘤抗原标记物的研究现状

胆管癌生发于胆管上皮细胞,并以浸润性的方式向周围胆管和肝组织扩散,导致肿瘤境界不清。大体上分为硬化型、结节型、乳头状和弥散浸润型;组织类型以腺癌最多见,少见的有腺鳞癌、黏液腺癌、鳞状细胞癌等。胆管癌常合并肝胆管结石等,病理改变极为复杂,致使以往术前诊断率较低,且不易与肝细胞癌、肝胆管结石等鉴别。胆管癌与其它转移性癌之间的鉴别也是病理诊断中经常遇到的问题。     

胆管内乳头状粘液瘤诊治

正胆管导管内乳头状黏液性肿瘤(intraductal papillary mucinous neoplasm of the bile tract,IPMN-B)是胆管内乳头状肿瘤(intraductal papillary neoplasm of the bile tract,IPNB)的一种病理类型,近些年才逐步被临床、影像及病理科医生所认识,特别是2010年世界卫生组织(WHO)消化系统肿瘤分类中将IPNB作为一组独立的疾病列出之后~([1])。IPNB     

胆管内乳头状黏液性肿瘤的手术治疗及预后分析

目的 探讨胆管内乳头状黏液性肿瘤（intraductal papillary mucinous neoplasm of the bile tract,IPMN-B）的手术治疗要点及预后特点。方法 回顾性分析郴州市第一人民医院2009年1月至2021年1月26例胆管内乳头状黏液性肿瘤患者手术及随诊的临床资料。结果 26例患者中男5例,女21例,平均年龄（62.0±7.3）岁。手术方式：左肝外叶切除+胆管探查术2例,左肝外叶切除+胆肠内引流术2例,左半肝切除（含尾状叶）+胆肠内引流术16例,右半肝+胆管探查术3例,肝门部胆管及肝IVb段切除+胆肠吻合术1例,肿瘤活检+胆管探查姑息性T管外引流术2例。术后病理诊断：胆管内乳头状黏液腺癌7例,胆管内乳头状黏液腺瘤18例,无明确肿瘤病变者1例。术后并发症：胆漏2例,肺部感染5例,消化道出血2例,伤口感染3例,均经治疗痊愈出院。随访期间T管黏液阻塞感染1例,术后6年复发再行手术治疗1例,术后3年复发合并胃瘘及消化道出血1例、术后45 d残余病灶致胆管炎死亡1例,其余预后良好。结论 IPMN-B手术治疗安全有效,精准肝叶段切除可以防止复发,且要注意黏液性肿瘤多发及胆管外侵蚀播散等特性。     

弥漫型胆管癌临床病理学特征及外科治疗

胆管癌分为肝内、肝门、远端及弥漫型四种。弥漫型胆管癌是指肿瘤浸润较广泛或浸润全部肝外胆管,有两种类型,即浅表扩散性和弥漫浸润性。浅表扩散是指胆管癌从主瘤灶沿黏膜扩散>20 mm者,常见于胆管乳头状癌及其他分化好的腺癌中,恶性程度较低,预后较好。弥漫浸润是指胆管癌累及从肝门至远端的全部肝外胆管,病理学分期晚,预后差。应用MRI、多层螺旋CT以及胆管镜等检查,大多数病人可准确诊断胆管癌的大体类型及胆管累及的程度,特别是在浅表扩散性胆管癌中明确胆管黏膜累及的范围至关重要,这有助于设计精准的手术方案。弥漫型胆管癌需行包括大范围肝切除的肝胰十二指肠切除术者,术前大多需行胆道引流和门静脉栓塞,以增加手术的安全性及防止术后发生肝功能衰竭。     

胆管导管内乳头状瘤误诊为肝门胆管癌一例

<正>胆管导管内乳头状瘤(intraductal papillary neoplasm of the bile ducts,IPNB)是一种起源于胆管上皮的导管内生长的肿瘤,由乳头状和绒毛状结构组成,并伴有纤细的纤维血管覆盖,其特点为胆管黏膜上皮细胞呈乳头状增生,目前认为IPNB是一种癌前病变,通常具有高度恶性潜能^[1]。IPNB临床上较罕见,发病较为隐匿,临床症状、     

胆管内乳头状黏液性肿瘤诊治进展

正2010年世界卫生组织(WHO)消化系统肿瘤分类中将胆管内乳头状瘤(intraductal papillary neoplasm of the bile tract,IPNB)作为一组独立的疾病列出~([1])。IPNB分为有分泌功能和无分泌功能肿瘤。其中有分泌功能者占28%~37%,称之为胆管内乳头状黏液性瘤(intraductal papillary mucinous neoplasm of the bile tract,BT-IPMN),无分泌功能     

第5版WHO消化系统肿瘤分类解读：胆道系统肿瘤的更新及进展

第5版WHO消化系统肿瘤分类中，胆道系统肿瘤更新后总体改变不多。在良性肿瘤及癌前病变部分，将胆管上皮内瘤变的3级分类系统改为高级别与低级别两个级别。同时新增胆囊与胆管导管乳头状肿瘤伴相关浸润性癌。肝内胆管肿瘤分类中，新增胆管腺瘤和胆管腺纤维瘤。这两类疾病在以往版本，常在与胆管癌鉴别诊断中提到。第5版WHO消化系统肿瘤分类的肝内胆管癌分为小胆管亚型和大胆管亚型，并不再推荐使用胆管细胞癌和细胆管细胞癌的名称。肝外胆管癌首次列为单独部分，除常见的组织学类型外，还包括鳞状细胞癌、腺鳞癌和未分化癌。另外，新版分类中增加了不少分子病理学相关的内容，为肿瘤的精准分类及诊断提供了依据。     

肝细胞癌伴胆管癌栓诊治进展

肝细胞癌可侵犯肝内外胆管形成胆管癌栓,其易与胆总管结石、胆管癌等疾病相混淆。目前,肝细胞癌合并胆管癌栓的诊断及鉴别诊断主要依靠超声、CT、磁共振等影像学检查。在肝细胞癌伴胆管癌栓治疗方面,外科手术切除已成为主要治疗方式。尽管有文献报道肝细胞癌伴胆管癌栓的病人在接受根治性手术后能达到与不伴癌栓的病人相似的生存结局,但总体上此类病人的预后仍不良。手术治疗与胆管引流、经动脉化疗栓塞、射频消融等非手术疗法的综合应用或能提高此类病人的远期预后,在肝细胞癌伴胆管癌栓的治疗中具有良好应用前景。     

胆管内乳头状粘液瘤诊治

正胆管导管内乳头状黏液性肿瘤(intraductal papillary mucinous neoplasm of the bile tract,IPMN-B)是胆管内乳头状肿瘤(intraductal papillary neoplasm of the bile tract,IPNB)的一种病理类型,近些年才逐步被临床、影像及病理科医生所认识,特别是2010年世界卫生组织(WHO)消化系统肿瘤分类中将IPNB作为一组独立的疾病列出之后~([1])。IPNB可分为有分泌功能的和无分泌功能的两种类型,其中有分泌功能者,即IPMN-B占28%～37%,无分泌     

Similarities and differences between intraductal papillary tumors of the bile duct with and without macroscopically visible mucin secretion

Intraductal papillary neoplasms of the bile duct (IPNB) have been recently proposed as the biliary counterpart of intraductal papillary mucinous neoplasms of the pancreas (IPMN-P). However, in contrast to IPMN-P, IPNB include a considerable number of the tumors without macroscopically visible mucin secretion. Here we report the similarities and differences between IPNB with and without macroscopically visible mucin secretion (IPNB-M and IPNB-NM). Surgically resected 27 consecutive cases with IPNB were divided into IPNB-M (n=10) and IPNB-NM (n=17), and their clinicopathologic features were examined. Clinically, both tumors were similar. Pathologically, the most frequent histopathologic types were pancreatobiliary in IPNB-NM and intestinal in IPNB-M. Various degrees of cytoarchitectural atypia within the same tumor were exhibited in 8 IPNB-M, but only 3 in IPNB-NM. Although the tumor size was similar, 9 IPNB-NM were invasive carcinoma, whereas all but 1 IPNB-M with carcinoma were in situ or minimally invasive. Immunohistochemically, positive MUC2 expression was significantly more frequent in IPNB-M than in IPNB-NM, whereas MUC1 tended to be more frequently expressed in IPNB-NM compared with IPNB-M. Among IPNB-NM with positive MUC1 expression, 3 had negative MUC2 and MUC5AC expressions. These tumors showed a tubulopapillary growth with uniform degree of cytoarchitectural atypia. All IPNB-M were negative for p53, and the frequency of positive p53 protein in IPNB-NM was at the middle level of that in IPNB-M and nonpapillary cholangiocarcinoma. In conclusion, IPNB-M showed striking similarities to IPMN-P, but IPNB-NM contained heterogeneous disease groups.     

Multicentric recurrence of intraductal papillary neoplasms of bile duct in the remnant intrahepatic bile duct after curative resection

IntroductionThere have been few reports on the prognosis of patients with intraductal papillary neoplasms of the bile duct (IPNB). Here we report a case of IPNB in a patient with early-stage carcinoma who had multicentric recurrence in the remnant hepatic bile duct after curative resection.Case presentationA 78-year-old man with hepatic dysfunction and cholestasis was referred to our hospital. Preoperative imaging studies revealed the presence of papillary tumors in the left hepatic duct and common hepatic duct, while no tumor lesions were detected in the right hepatic duct. This patient underwent left hepatectomy, extra-hepatic bile duct resection with biliary reconstruction, and regional lymphnode dissection. On the basis of pathological examination, this patient was diagnosed with multiple IPNB with early-stage adenocarcinoma with negative surgical margin. Postoperative work-up was periodically performed, indicating no evidence of recurrence, while the patient had sustained hepatic dysfunction, cholestasis, and repetitive cholangitis since the early postoperative period. Finally, recurrence in the remnant intrahepatic bile duct of the posterior segment was revealed by double balloon enteroscopy at 29 months after surgery. At 34 months after surgery, internal drainage stents were replaced in both endoscopic and percutaneous manners within the relapsed intrahepatic bile ducts to address repetitive cholangitis. These procedures enabled the patient to remain asymptomatic until death at 41 months after surgery.DiscussionMulticentric recurrence in the remnant intrahepatic bile duct after surgery may occur in IPNB patients with multiple lesions. An endoscopic approach may be useful in such cases, not only in the diagnosis of remnant intrahepatic bile duct recurrence but also for palliation of symptoms.     

肝内胆管乳头状瘤13例CT诊断分析

目的分析肝内胆管乳头状瘤（IPNB）的cT表现,探讨CT对其的诊断价值。方法回顾性分析13例经手术及病理证实的IPNB的cT和临床资料。结果13例IPNB中发生于右叶肝内胆管6例,左叶肝内胆管4例,近肝门处胆管3例。11例表现为扩张的肝内胆管内形态多变的结节或肿块,3例单发,8例多发;CT平扫呈不均匀低密度,边界欠清,动态增强呈轻～中度持续强化,强化不均匀,边界变清晰,病灶周边仍见较完整的胆管壁。另外2例CT平扫表现为中度扩张的胆管内壁毛糙如绒毛状,平扫较胆汁呈稍高密度,增强轻度强化。13例均见肝内胆管呈弥漫性或局限性不同程度扩张,5例肿瘤所在胆管呈“动脉瘤”样扩张。结论IPNB的cT表现具有一定特征,CT对其诊断有较大价值。     

Impact of Macroscopic Morphology, Multifocality, and Mucin Secretion on Survival Outcome of Intraductal Papillary Neoplasm of the Bile Duct

Background

Intraductal papillary neoplasms of the bile duct (IPNB) are relatively rare disease with favorable prognosis. The authors investigated clinicopathologic characteristics and prognostic factors of IPNB in viewpoint of macroscopic morphology and multiplicity.

Methods

Data were collected from 84 patients who underwent surgery at Seoul National University Hospital with diagnosis of IPNB from 2000 to 2009.

Results

Median follow-up was 41.8 months and 75 (89.3 %) had invasive cancer. Tumors were confined to the bile duct in 45 patients (53.6 %) and 8 (9.5 %) had lymph node metastasis. Curative resection was achieved in 70 patients (89.3 %). Mucin secretion was identified in 23 (28.0 %) and 43 (51.2 %) had multiple tumors. Multiple IPNB had poor prognosis compared with single IPNB (5-year survival rate 50.7 vs. 85.9 %; P?=?0.011). Positive resection margin (P?=?0.046) and multiplicity (P?=?0.038) were independent prognostic factors of IPNB after multivariate analysis. Mucin secretion had no impact on survival outcome (P?=?0.595). The disease-free survival rate was significantly lower in multiple IPNB compared with single IPNB (5-year disease free survival rates 36.1 vs. 74.1 %; P?=?0.026).

Conclusion

Multiplicity is a common feature of IPNB and has a negative impact on prognosis. Current WHO classification for IPNB needs consideration for macroscopic morphology and multiplicity considering its prognostic impact of IPNB.     

肝胆管结石合并胆管癌的诊治分析

目的总结肝胆管结石合并胆管癌的诊治经验。方法回顾性分析46例肝胆管结石合并胆管癌患者的临床资料,其中18例行根治性手术,28例行姑息手术。结果病理诊断为胆管腺癌36例,粘液癌10例。姑息组中有3例仅行活检术,术后1月死于肝功能衰竭。术后发生胆瘘1例,肝断面胆漏2例,伤口感染5例,均经保守治愈,2例伤口裂开经二期缝合治愈。随访43例,根治组平均存活26(13～45)个月,姑息治疗组平均存活10(5～14)个月。根治组术后1、2、3年生存率分别为94.4%、55.6%、33.3%,姑息组术后1年生存率为39.3%,无二年生存。结论肝胆管结石反复炎性刺激可并发胆管癌,根治性切除可延长生存期,姑息手术可提高生存质量;提高肝胆管结石合并胆管癌疗效的关键是争取早期根治性治疗肝胆管结石。     

肝门部胆管癌的临床诊断与手术治疗分析

目的探讨肝门部胆管癌的临床诊断方法及两种不同手术方法的预后。方法选取2002年12月至2008年3月就诊的肝门部胆管癌患者43例作为研究对象,回顾性分析所有患者的临床表现、影像学检查结果、手术方式、生存率等临床资料,所有对象按手术方法分为根治性切除术组（22例）和姑息性切除术组（21例）,对比分析两组间患者的术后并发症发生率,1、3、5年生存率有无统计学差异。结果临床表现以黄疸最为常见,占86．0％,其次是尿黄（81．4％）和皮肤瘙痒（72．1％）;经过常规超声、CT及MRI联合检查,根治性切除术组和姑息性切除术组对肝门部胆管癌检出率分别为90．9％和95．2％;MRI诊断阳性率明显高于超声诊断阳性率（χ^2=7．379,P〈0．01）。根治性切除术组患者并发症发生率明显高于姑息性切除术组（χ^2=14．321,P〈0．01）,1、3、5年生存率也明显高于姑息性切除术组（r=6．018、χ^2=2．842、r=17．483,P〈0．05）。结论对于肝门部胆管癌患者,MRI诊断阳性率明显高于超声,采取超声联合CT或MRI可提高早期诊断率;根治性切除术治疗肝门部胆管癌,能够提高此类患者的远期存活率。     

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??Clinicopathological features and surgical treatment of diffuse cholangiocarcinoma WU Zhi-yong, Department of Gastrointestinal Surgery??Ren Ji Hospital??School of Medicine??Shanghai Jiao Tong University, Shanghai 200127, China
Abstract Cholangiocarcinoma has been classified as intrahepatic, perihilar, distal and diffuse. Diffuse cholangiocarcinoma exhibits an extensive ductal spread invading from the hepatic hilus to the lower bile duct, which has two types, namely the superficial spreading and diffuse infiltrating type. Superficial spreading cholangiocarcinoma is defined as mucosal extension of more than 20mm from the main lesion, which is mostly observed in papillary carcinoma or other well differentiated adenocarcinomas of the bile duct. Superficial spreading cholangiocarcinoma is with low malignancy and comparatively good prognosis. Diffuse infiltrating cholangiocarcinoma demonstrates that invading of the tumor involves whole extrahepatic bile duct, whose pathological staging is often late and the prognosis is poor. The gross type of cholangiocarcinoma and the invasive extent of tumor in most cases can be accurately evaluated by MRI??Multidetector Row CT??MDCT??and choledochoscope, etc. It’s especially important to evaluate the extension of the mucosa in the superficial spreading cholangiocarcinoma, which will help the surgeons to design a precise surgery. Most of the patients suffered from diffuse cholangiocarcinoma need to undergo hepatopancreatoduodenectomy including major liver resection. To prevent from postoperative liver failure and improve surgical safety??it is necessary that the most patients receive biliary drainage and portal vein embolization before the operation.     

胆管癌的诊断与治疗研究进展

胆管癌具有较高的恶性程度和较低的手术切除率,加之术后辅助化疗和靶向治疗药物并不成熟,导致胆管癌患者的病死率较高,长期预后较差。随着目前的临床诊疗水平的提高、术前评估和外科技术的发展,胆管癌的诊断和治疗已取得了一些发展进步;胆管癌早期缺乏特异性表现,因此患者被诊断时已失去R0切除机会,早期诊断可提高患者R0切除及术后长期生存率,可通过对比增强超声、增强磁共振、正电子发射计算机断层显像等提高诊断准确性。在治疗上胆管癌仍以综合治疗为主,对于可切除胆管癌争取行R0切除,发生远处转移的胆管癌可结合新辅助化疗后根据复查情况决定是否可行手术治疗、术后联合化疗、分子靶向治疗、立体定向放射治疗等综合方案以期提高患者长期生存率。本文就胆管癌的发病机制、诊断及治疗的新进展作一综述。     

Intraductal papillary neoplasm of the bile duct: a case report

Intraductal papillary neoplasm of the bile duct (IPNB) is a rare variant of bile duct tumors, characterized by papillary growth within the bile duct lumen and is regarded as a biliary counterpart of intraductal papillary mucinous neoplasm (IPMN) of the pancreas. IPNBs are mainly found in patients from Far Eastern areas, where hepatolithiasis and clonorchiasis are endemic. The Western experience, however, remains limited. In this article, we report a 56-year-old man, referred to our hospital because of deranged liver function tests. Further imaging modalities showed a cystic lesion of 9?cm diameter, arising from the left hepatic duct. Inlying was a heterogeneous, lobulated mass. The patient underwent a left hemihepatectomy and adjuvant chemotherapy. Despite recent advanced technologies, diagnosis of IPNB is still challenging, especially in western countries due to its rarity. Early identification and resection of lesions, even in asymptomatic or minimally symptomatic patients, are however important prognostic factors.