磁共振胰胆管造影检查在肝门部胆管癌诊治中的应用

 【摘要】　目的　探讨磁共振胰胆管造影（MRCP）在肝门部胆管癌术前评估中的价值。方法　采用改良的手术标准，选择57例有潜在手术切除可能的肝门部胆管癌患者，术前进行MRCP影像学评估, 并与手术和病理对比。结果　MRCP术前定性准确率为100 %，分型准确性为93 ％（53／57）；肝管汇合部变异8例，变异率26.7 ％，有些变异对手术有利；左、右肝管增长，其中以左肝管尤其明显，这对胆肠吻合口的选择有利；术前MRCP检查显示，胆管癌病变上缘至胆管二级分支之间胆管长度＞0.5 cm或存在胆道变异的部分Ⅳ型肝门部胆管癌患者，手术切除率及根治率均明显提高，与其他3型之间差异无统计学意义。结论　MRCP可对肝门部胆管癌进行较准确的术前定性和分型；术前MRCP显示肝内二级胆管支及肝管汇合部变异对制定肝门部胆管癌，尤其是Ⅳ型肝门部胆管癌的外科手术方案有重要意义，不仅能提高切除率和根治率，而且有利于选择合适的胆肠吻合方式，可避免术中胆道误损伤。     

25例巨块型原发性肝癌切除体会

目的：探讨巨块型肝癌手术切除的可行性及技术要点.方法：回顾性分析2007年-2012年手术切除的25例巨块型原发性肝癌病例特点、手术方法及预后情况.结果：6例行肝左外叶切除,2例行左半肝切除,3例行肝中叶切除,4例行右半肝切除术,10例行肝叶不规则切除术.手术切除率100％.其中,门静脉切开取栓、门静脉化疗泵植入5例,胆总管切开取栓、胆总管T管引流1例.术中根据肿瘤部位选择性阻断肝脏血流.平均手术时间280±125min,术中出血720±260ml,无手术死亡.术后21例有不同程度腹水,胸腔积液12例,肺不张4例,乳糜漏1例,肝功能衰竭1例.无住院死亡病例.术后随访1年生存率59.6％,3年生存率29.8％,5年生存率19.5％.结论：在严格把握手术适应证前提下,选择合适的肝脏血流阻断方法及断肝方法,保证余肝体积,巨块型原发性肝癌的手术治疗是可行的.     

Surgical treatment of cancer of the biliary tract and gall bladder

With the advances in various kinds of diagnostic methods and improvement of operative technique, operations for cancer in biliary tract have recently increased, however, the prognosis has been unsatisfied. During the past 25 years, 495 cases with carcinoma of biliary tract (ca of gall bladder 175 cases, ca of bile duct 201, and ca of papilla vater 105) were operated. The resectability rate was 66% (62% in gall bladder, 62% in bile duct, 84% in papilla vater). Among the lesions in bile duct, the resectable rate in lower bile duct was better than the other site in bile duct. The late results in ca of bile duct, especially in the lesion of upper bile duct were still poor, however, the 5-years survival rate in ca of gallbladder or papilla vater carcinoma was 61% or 56%, respectively. For the further improvement of the surgical results, the sufficient resection of the intrahepatic bile duct in ca of bile duct should be performed, and in gall bladder carcinoma when tumor extends into the neck of gall bladder, bile duct should be resected. In advanced carcinoma, the appropriate hepatectomy or bile duct resection should be considered in proportion to the operative influence and the extension of the tumor.     

Principles of surgical resection in hilar cholangiocarcinoma

The aim of this article is to describe the surgical techniques for the treatment of hilar cholangiocarcinoma(HC).Resection with microscopically negative margin(R0) is the only way to cure patients with HC.Today,resection of the caudate lobe and part of segment Ⅳ,combined with a right or left hepatectomy,bile duct resection,lymphadenectomy of the hepatic hilum and sometimes vascular resection,is the standard surgical procedure for HC.Intraoperative frozen-section examination of proximal and distal biliary margins is necessary to confirm the suitability of resection.Although lymphadenectomy probably has little direct effect on survival,inaccurate staging information may influence post resection treatment recommendations.Aggressive venous and arterial resections should be undertaken in selected cases to achieve a R0 resection.The concept of "no-touch proposed" in 1999 by Neuhaus et al combine an extended right hepatectomy with systematic portal vein resection and caudate lobectomy avoiding hilar dissection and possible intraoperative microscopic dissemination of cancer cells.More recently minor liver resections have been proposed for treatment of HC.As the hilar bifurcation of the bile ducts is near to liver segments Ⅳ,Ⅴ and Ⅰ,adequate liver resection of these segments together with the bile ducts can result in cure.     

Surgical strategy for bile duct cancer: Advances and current limitations

The aim of this review is to describe recent advances and topics in the surgical management of bile duct cancer. Radical resection with a microscopically negative margin (R0) is the only way to cure cholangiocarcinoma and is associated with marked survival advantages compared to margin-positive resections. Complete resection of the tumor is the surgeon's ultimate aim, and several advances in the surgical treatment for bile duct cancer have been made within the last two decades. Multidetector row computed tomography has emerged as an indispensable diagnostic modality for the precise preoperative evaluation of bile duct cancer, in terms of both longitudinal and vertical tumor invasion. Many meticulous operative procedures have been established, especially extended hepatectomy for hilar cholangiocarcinoma, to achieve a negative resection margin, which is the only prognostic factor under the control of the surgeon. A complete caudate lobectomy and resection of the inferior part of Couinaud's segment IV coupled with right or left hemihepatectomy has become the standard surgical procedure for hilar cholangiocarcinoma, and pylorus-preserving pancreaticoduodenectomy is the first choice for distal bile duct cancer. Limited resection for middle bile duct cancer is indicated for only strictly selected cases. Preoperative treatments including biliary drainage and portal vein embolization are also indicated for only selected patients, especially jaundiced patients anticipating major hepatectomy. Liver transplantation seems ideal for complete resection of bile duct cancer, but the high recurrence rate and decreased patient survival after liver transplant preclude it from being considered standard treatment. Adjuvant chemotherapy and radiotherapy have a potentially crucial role in prolonging survival and controlling local recurrence, but no definite regimen has been established to date. Further evidence is needed to fully define the role of liver transplantation and adjuvant chemo-radiotherapy.     

What is the current state-of-the-art imaging for detection and staging of cholangiocarcinoma?

Cholangiocarcinoma is an adenocarcinoma that arises from the bile duct epithelium and is the second most common primary hepatobiliary cancer, after hepatocellular cancer, with approximately 2,500 cases annually in the U.S. However, cholangiocarcinoma remains a relatively rare disease, accounting for <2% of all human malignancies. Although the entire biliary tree is potentially at risk, tumors involving the biliary confluence or the right or left hepatic ducts (hilar cholangiocarcinoma) are most common and account for 40%-60% of all cases. Most patients present with advanced disease that is not amenable to surgical treatment. The median survival time for patients with intrahepatic cholangiocarcinoma without involvement of the hilum varies among centers from 18-30 months. The median survival time for patients with perihilar cholangiocarcinoma is slightly less, varying from 12-24 months. Despite the overall poor prognosis, survival after surgical treatment of hilar cholangiocarcinoma has improved during the past 10-15 years. This review highlights the imaging features of cholangiocarcinoma, with particular emphasis on the imaging techniques that can best assess tumor resectability and guide the surgeon regarding the potential extent of resection required in operable candidates.     

Extended right hepatectomy for hilar cholangiocarcinoma with resection of the left hepatic duct prior to hepatic resection

Extended hepatectomy for hilar cholangiocarcinoma results in high operative or in-hospital mortality rates despite of the recent progress in perioperative management. As a new procedure to prevent postoperative hepatic failure in hilar cholangiocarcinoma infiltrating predominantly the right hepatic duct, we devised a combination of extended right lobectomy plus caudal lobectomy with resection of the left hepatic duct prior to hepatic resection by utilizing intraoperative cholangiography, and applied the procedure to a 70-year-old patient. He had a favorable postoperative course and remains recurrence-free at 4 years after operation. This is a procedure for confining the extent of hepatectomy to the minimum necessary, aiming at curative resection of hilar cholangiocarcinoma.     

Pathological features and surgical outcome of pancreaticobiliary maljunction without dilatation of the extrahepatic bile duct

In cases of pancreaticobiliary maljunction without dilatation of the extrahepatic bile duct (undilated PBM), preventive cholecystectomy is performed because there is a high incidence of gallbladder cancer as compared to cases of PBM with dilatation of the extrahepatic bile duct (dilated PBM). However, it is still controversial whether resection of the extrahepatic bile duct should also be performed in patients with undilated PBM. Accordingly, we analyzed pathological findings, postoperative complications and a long-term prognosis in 19 patients with undilated PBM to clarify the possibility of the bile duct cancer. In undilated PBM, hyperplasia was significantly recognized in the gallbladder as compared to the bile duct (p=0.0238), while no significant differences were found in other epithelium. Atypical epithelium and hyperplasia in gallbladder mucosa of undilated PBM were significantly recognized as compared to cases of pancreas or biliary tract cancer without PBM (p=0.0035, p=0.0019, respectively), while no significant differences were recognized in any kind of epithelium of the bile duct. In 14 cases of undilated PBM with preservation of the extrahepatic bile duct, the postoperative observation period was from 1 year and 5 months to 18 years and 10 months (mean: 8.3 years). One of the 5 patients with gallbladder cancer died 2 years and 6 months after surgery due to the cancer recurrence, while the remaining 13 patients had no complications such as liver dysfunction, cholangitis or remnant bile duct cancer, and the patients have survived in good health. These findings indicate that preventive bile duct resection is not necessary in patients with undilated PBM.     

肝门部胆管癌的外科治疗:291例回顾

目的 分析一个单位1986年-2002年间治疗肝门部胆管癌291例的经验。方法 回顾1986年-2002年在解放军总医院肝胆外科治疗291例肝门部胆管癌的纪录,病例分为2组:Ⅰ组:1986年1月-1999年1月,共157例;Ⅱ组为1999年2月-2002年6月,共134例。外科治疗手段包括根治性切除术、姑息性切除术或内、外胆道引流术,主要是依据手术中所发现的病理情况决定。根治性切除术的标准是指切除的边缘病理上未发现残留癌细胞者。根治性切除率在两组分别为37.6％和41.2％。无切除术后30天内死亡。随访结果是通过信件、电话及门诊获得,随访率为88.8％。结果 在我国,肝外胆管癌是并非少见的疾病,近年来手术治疗的病例数有增多倾向。然而,由于肿瘤居于肝门部胆管的深在位置,所以根治性切除手术有困难,甚至联合肝切除亦难以达到根治目的,因而在两组病例中,根治性切除率分别仅为37.6％和41.2％。在第Ⅰ组中,有4例病人于切除术后长期无瘤生存,5年以上生存率为13.3％;另有2例病人亦生存达5年以上,但癌复发,现仍在接受进一步治疗。在第Ⅱ组中尚未有5年生存者,3年生存率为13.6％。结论 肝门部胆管癌是多态性的疾病,只有极少数表现为较“良性”的倾向,而绝大多数则于手术切除后易于复发,虽然手术似乎是已达治愈性。切除性治疗,甚至是姑息性切     

The long-term survival of patients with carcinoma of the main hepatic duct junction

Carcinoma of the main hepatic duct junction tends to invade extensively the bile ducts and hepatic parenchyma, although dissemination is rarely seen. Therefore, extensive resection of the bile ducts combined with hepatic resection is the procedure of choice for treating this disease. From January 1973 to January 1987, 23 of 41 patients underwent resection, giving a resectability rate of 56%. One patient died postoperatively, yielding an operative mortality rate of 4.3%. The 5-year actuarial survival rate is 29.8%. Three patients are now alive and well 6 years and 9 months, 5 years and 10 months, and 5 years and 5 months after the operation. One additional patient who underwent resection in an affiliated hospital has done well for 8 years and 8 months. The results from these four patients treated by curative resection support a strategy featuring curative resection with aggressive surgery.     

The caudate lobe of the liver: implications of embryology and anatomy for surgery

The anatomy of the caudate lobe has technical and possibly oncologic implications for surgeons. The complex anatomy of the lobe is clarified by embryologic and anatomic analysis. This posterior sector is embryonically and anatomically independent of the right and left liver and the main portal fissure. The caudate lobe represents the only part of the liver that is in contact with the vena cava, except at the entrance of the main hepatic veins into the vena cava, and provides an anastomosis between the hepatic veins and vena cava. The entire caudate lobe is a single anatomic segment that is defined by the presence of portal venous and hepatic arterial branches, which supply the lobe, draining biliary ducts, and hepatic veins. Because no separate veins, arteries, or ducts can be defined for the right paracaval portion of the posterior liver and because pedicles cross the proposed division between the right and left caudate, the concept of segment IX is abandoned. The significance of caudate anatomy is reflected in the increase in the frequency and safety of major hepatic resection for primary and metastatic tumors in the liver. Right hepatic lobectomy routinely involves resection of the right portion of the caudate lobe (C. Couinaud, unpublished data, 1999). In the case of hilar bile duct cancer, which may extend into the dorsal ducts (especially the right lateral duct), partial or total caudate lobectomy is often necessary for complete extirpation of the tumor. Isolated caudate lobectomy can be performed for hepatocellular carcinoma that arises in the caudate lobe or for other tumors that arise in the lobe. The caudate lobe can be resected as part of the donor liver in preparation for a living related donor transplantation. Knowledge of the surgical anatomy of the caudate lobe is an essential part of the repertoire for surgeons who perform liver transplants or treat hepatobiliary cancer.     

Liver resection in malignant disease

As more surgeons become familiar with the techniques of hepatic resection and the mortality and morbidity decrease, the indications for resection of malignant disease within the liver broadens. The preoperative assessment of malignant liver lesions, as well as the definition of resectability, are outlined. Indications for operative intervention as well as the results obtained are covered. The personal experience of the authors at the Royal Postgraduate Medical School Hepatobiliary Unit, Hammersmith Hospital, in dealing with malignant lesions of the liver is detailed with respect to procedures performed and postoperative morbidity and mortality. Hepatocellular carcinoma, hilar cholangiocarcinoma, and metastatic colon carcinoma are discussed in detail. The authors' experience with each of these diseases is presented.     

A case of curative resected hilar bile duct cancer with hepatic artery and portal vein reconstructions

The indication for surgical resection due to hilar bile duct cancer (BDC) with vessel reconstructions is still controversial. We report herein a successfully resected case due to hilar BDC with hepatic artery (HA) and portal vein (PV) reconstructions using autograft from a resected liver. A 57-year-old woman was diagnosed as hilar BDC, and computed tomography showed a tumor invaded left and common hepatic duct, right and left HA, and left main branch of PV. Because the extrahepatic area of right HA was free from the tumor, we performed left hepatectomy and caudate lobectomy with HA and PV reconstructions. We used auto left hepatic vein graft from the resected liver for PV reconstruction, because there was no appropriate size vein graft, e.g. inferior mesenteric vein. The patient is alive without any evidence of recurrence for 8 months after the surgery.     

原发性肝癌合并胆管癌栓的外科治疗

目的：提高对原发性肝癌合并胆管癌栓的认识，探讨其诊断、治疗方法及疗效。方法：回顾性分析我院1998年1月至2004年1月间收治的42例原发性肝癌合并胆管癌栓的临床资料，施行根治性手术26例，其中18例肝叶切除＋胆总管切开取栓、T管引流术；8例肿瘤局部切除＋胆总管切开取栓、T管引流术；姑息性手术16例，其中6例胆总管切开取栓、T管引流术，10例胆总管切开取栓、T管引流术＋患侧肝动脉结扎术（其中4例＋门静脉DDS泵置入术）。均获得病理诊断，肝细胞癌32例（76．2％）。结果：根治性手术及姑息性手术1年、3年、5年生存率分别为65．4％（17／26），42．3％（11／26），15．4％（4／26）及18．8％（3／16）、6．3％（1／16），0（0／16）；总的1年、3年、5年生存率分别为47．6％（20／42），28．6％（12／42），9．5％（4／42）。结论：外科治疗明显改善患者生活质量，提高了生存时间，而根治性手术是原发性肝癌合并胆管癌栓的积极有效方法。     

Application of endoscopic nasobiliary cutting in the treatment of hilar cholangiocarcinoma

Objective The aim of the study was to study the clinical efficacy and prognosis of endoscopically cutting the nasobiliary duct and leaving its residual segment as a biliary stent in the treatment of hilar cholangiocarcinoma (HC). Methods The clinical data of 55 patients with HC treated by endoscopic biliary drainage at the Gastrointestinal Endoscopy Center of our hospital (Renmin Hospital of Wuhan University, China) from August 2017 to August 2019 were retrospectively analyzed. According to different drainage schemes, patients were divided into the endoscopic nasobiliary cutting group (n = 26) and the endoscopic retrograde biliary drainage (ERBD) group (n = 29). The postoperative liver function indexes, incidence of postoperative complications, median patency period of stents, and median survival time of patients were compared between the two groups. Results Liver function indexes (total bilirubin, direct bilirubin, alanine aminotransferase, aspartate aminotransferase, alkaline phosphatase, gamma-glutamyl transpeptidase) were significantly decreased in 55 patients a week postoperaticely (P < 0.05), and decreases in liver function indexes in the endoscopic nasobiliary cutting group were more significant than those in the ERBD group (P < 0.05). The incidence of biliary tract infection in the endoscopic nasobiliary cutting group was significantly lower than that in the ERBD group (15.40％ vs. 41.4％, P < 0.05). In the endoscopic nasobiliary cutting subgroups, there were 1 and 3 cases of biliary tract infection in the gastric antrum cutting group (n = 21) and duodenal papilla cutting group (n = 5), respectively, and 0 cases and 2 cases of displacement, respectively; there was a statistically significant difference in terms of complications between the two subgroups (P < 0.05). The median patency period (190 days) and median survival time (230 days) in the nasobiliary duct cutting group were higher than those (169 days and 202 days) in the ERBD group, but there was no significant difference (P > 0.05). Conclusion The nasobiliary duct was cut by using endoscopic scissors in Stage Ⅱ after the bile was fully drained through the nasobiliary duct. The residual segment could still support the bile duct and drain bile. The reduction of jaundice and the recovery of liver enzymes were significant, and the incidence of biliary tract infection was low. Cutting off the nasobiliary duct at the duodenal papilla results in a higher incidence of biliary tract infection, and the residual segment of the nasobiliary duct is more likely to be displaced. Endoscopic nasobiliary-cutting drainage is an effective, simple, and safe method to reduce jaundice in the palliative treatment of HC.     

影像学检查对肝门部胆管癌可切除性评估的价值

目的 探讨影像学检查对肝门部胆管癌可切除性的评估价值。方法 对43例经术后病理组织学证实的肝门部胆管癌患者的CT及MRI资料进行回顾性分析,包括肿瘤的大小、胆管受侵犯的长度、肿瘤侵犯门静脉及肝动脉的程度、淋巴结转移及远处转移的情况、胆管受侵犯的范围及改良建议性T分期与可切除性的关系。结果 浸润型肝门部胆管癌的可切除率为8.3％,肿块型的可切除率为51.6％（P=0.017）。不同肿块大小和肿瘤浸润胆管的长度组别间可切除率的差异无统计学意义（P＞0.05）。Bismuth分型各型可切除率的差异无统计学意义（P＞0.05）。改良建议性T分期各期的可切除率的差异有统计学意义（P＜0.01）,且可切除率随T分期的增加而下降（P＜0.01）。结论 浸润型肝门部胆管癌的可切除率低于肿块型;肿块的大小和肿瘤浸润胆管的长度与肿瘤的可切除性均无关;改良建议性T分期较Bismuth分型在指导肝门部胆管癌的可切除性上更有价值。     

RESULTS OF HEPATECTOMY FOR HUGE PRIMARY LIVER CANCER

ＲＥＳＵＬＴＳＯＦＨＥＰＡＴＥＣＴＯＭＹＦＯＲＨＵＧＥＰＲＩＭＡＲＹＬＩＶＥＲＣＡＮＣＥＲＬｉＧｕｏｈｕｉ；李国辉；ＬｉＪｉｎｑｉｎｇ；李锦清；ＺｈａｎｇＹａｑｉ；张亚奇；ＣｕｉＳｈｕｚｈｏｎｇ；崔书钟；ＹｕａｎＹｕｎｆｅｉ；元云飞（ＴｕｍｏｒＨｏｓ...     

肝部分切除与肝十二指肠韧带骨髂化治疗肝门部胆管癌

Objective To summarize the surgical experience of partial hepatectomy with skeletonization of the hepatoduodenal ligament in the treatment of hilar cholangiocarcinoma.Methods Between Jan.1999 and Dec,2001,67 consecutive patients with hilar cholangiocarcinoma underwent surgical exploration at the Second Military Medical University,Eastern Hepatobiliary Surgery Hospital.The clinical data of these patients were reviewed.Results Of the 67 patients,65(97%) underwent surgical resection.Fourty-nine patients(73%) received curative resection:22 skeletonization resection(SR) and 27 SR combined with partial hepatectomy.In 16 patients(9%) with curative resection the tumor margin was histologically postive and the resection was therefore considered palliative.The tumors were classified according to Bismuth with SR was type Ⅱ(17cases),various types of partial hepatectomy with SR was type Ⅲ and type IV.Right lobectomy with right caudate lobectomy was indicated in type Ⅲ(6cases),left lobectomy with complete caudate lobectomy in type Ⅲb(15cases),right loobectomy with complete caudate lobectomy(3 cases),left lobectomy with complete caudate lobectomy(9 cases) and quadrate lobectomy(2 cases)in type IV.SR and left lobectomy with complete caudate lobectomy was successfully performed in 2 patients(3%) who had undergone palliative biliary resection and cholangiojejunostomy before.Eight patients(12%) had local resecton of the tumor with Roux-en-Y hepaticojejunostomy reconstruction using intrahepatic stents.Two patients(3%) had palliative biliary drainage.Combined portal vein resection was performed in 13 patients(20%) and hepatic artery resection in 27 patients(40%) .Twenty-four atients(36%) had no postoperative complication,23 patients(34%) had minor complications only ,and the remaining 20 patients(30%) had major complications.Of the 20 patients with major complications,14 recovered,the remaining 6 patients died from hepatorenal failure with other organ failures,from myocardial infarction or from intraabdominal or gastrointestianl bleeding 7,12,14,42,57 or 89 days after surgery.The 30-day operative mortality was 4.5%.The mean survival of the patient with curative resecton was 16 months(range 1-32 months);for those undergong palliative resection mean survival was 7 months(range 1-14months).Conlusion Partial hepatectomy with SR for hilar cholangiocarcinoma can be performed with acceptable morbidity and mortality.For curative treatmet of hilar cholangiocarcinoma,caudate lobectomy is always recommended in Bismuth Ⅲ/IV.     

Major Hepatic Resection for Hilar Cholangiocarcinoma without Preoperative Biliary Drainage.

Hilar cholangiocarcinoma is a rare cancer in western countries but very high incidence in the northeast of Thailand. The only chance to cure is surgical resection. Preoperative biliary drainage (PBD) for improving liver function to decrease perioperative morbidity and mortality is claimed to be beneficial. To determine whether liver resection with hilar resection is a safe procedure in obstructive jaundice patients caused by hilar cholangiocarcinoma, the records of 30 consecutive patients undergoing surgery between May 1999 and May 2002 at Srinagarind hospital, Khon Kaen University, were retrospectively analyzed. Two patients died during hospitalization, an operative mortality of 6.7%. Survival was 33% at 1 year, 12% at 2 years,10% at 3 years and 6.7% at 4 years. In our experience, it is safe in most patients with obstructive jaundice due to hilar cholangiocarcinoma to perform liver resection without preoperative biliary drainage (PBD).     

Intraductal papillary neoplasm of the bile ducts: the clinical features and surgical outcome of 25 cases

BACKGROUND AND OBJECTIVES: Intraductal papillary neoplasm of the bile ducts (IPN-B) is considered an uncommon tumor. The purpose of this study was to evaluate the clinical, radiological, and histopathological characteristics of IPN-B, and its prognosis. METHODS: From October 1995 to August 2006, a retrospective analysis was made of 25 patients that underwent surgery for IPN-B. Clinical features and radiological, pathological, and operative findings were reviewed, and survival rates were determined. RESULTS: In five patients (20.0%), lesions were incidentally found. Radiologically, 23 of the 25 (92.0%) showed bile duct dilatation, bile duct dilatation with or without an intraductal mass, and cystic changes of bile ducts. Twenty three of the 25 patients underwent hepatic resection with or without extrahepatic bile duct resection. No in hospital mortality occurred. Median survival time of resected patients was 59.8 months and 1-, 2-, and 4-year survival rates were 90.5%, 84.0%, and 84.0%, respectively. All six patients with benign IPN-B remained alive at a mean of 26.2 postoperative months without recurrence. CONCLUSIONS: A diagnosis of IPN-B is usually made in patients with biliary dilatation by radiologic study. The prognosis of IPN-B, especially of the benign category, is excellent. Aggressive surgical resection is the treatment of choice for IPN-B.