Cystic micropapillary neoplasm of peribiliary glands with concomitant perihilar cholangiocarcinoma

We report a case of a 75-year-old man with cystic micropapillary neoplasm of peribiliary glands detected preoperatively by radiologic examination. Enhanced computed tomography showed a low-density mass 2.2 cm in diameter in the right hepatic hilum and a cystic lesion around the common hepatic duct. cholangiocarcinoma, right hepatectomy with caudate lobectomy and bile duct resection were performed. Pathological examination revealed perihilar cholangiocarcinoma mainly involving the right hepatic duct. The cystic lesion was multilocular and covered by columnar lining epithelia exhibiting increased proliferative activity and p53 nuclear expression; it also contained foci of micropapillary and glandular proliferation. Therefore, the lesion was diagnosed as a cystic micropapillary neoplasm of peribiliary glands and resembled flat branch-type intraductal papillary mucinous neoplasm of the pancreas. Histological examination showed the lesion was discontinuous with the perihilar cholangiocarcinoma. Immunohistochemistry showed the cystic neoplasm was strongly positive for MUC6 and that the cholangiocarcinoma was strongly positive for MUC5 AC and S100 P. These results suggest these two lesions have different origins. This case warrants further study on whether this type of neoplasm is associated with concomitant cholangiocarcinoma as observed in pancreatic intraductal papillary mucinous neoplasm with concomitant pancreatic duct adenocarcinoma.     

Intrahepatic biliary papilloma morphologically similar to biliary cystadenoma

A 37-year-old man presented complaining of epigastralgia. Abdominal ultrasonography revealed the presence of a papillary tumor (9 mm in diameter) in the cystic lesion (18 mm in diameter) in hepatic segment 4, which was accompanied by mild intrahepatic bile duct dilatation. Although abdominal computed tomography also showed the cystic lesion, it did not show papillary tumors inside the lesion. Endoscopic retrograde cholangiography showed the communication between the cystic lesion and the left hepatic duct. In addition, mucus was observed in the common bile duct. When transpapillary intraductal ultrasonography was performed through the left hepatic duct using a fine ultrasonic probe, a hyperechoic papillary and lobulated tumor was clearly shown in the cystic lesion. The wall of the cyst was smooth and there was no sign of tumor infiltration. Based on these findings, biliary cystadenoma was diagnosed and an extended left lobectomy was carried out. However, pathological findings postoperatively revealed that the lesion was a localized biliary papilloma, developing and extending to the intrahepatic duct. This case is rare and there have been no published reports describing a biliary papilloma morphologically similar to biliary cystadenoma.     

Mucinous Cystic Neoplasm of the Liver or Intraductal Papillary Mucinous Neoplasm of the Bile Duct? A Case Report and a Review of Literature

Mucinous cystic neoplasm of the liver (MCN-L) and intraductal papillary mucinous neoplasm of the bile duct (IPMN-B) are diagnoses that were classified by the World Health Organization in 2010 as mucin-producing bile duct tumors of the hepatobiliary system. The preoperative differential diagnosis between these two entities is difficult; the presence of a communication with the bile duct is usually considered as a typical sign of IPMN-B. However, the presence of an ovarian-like stroma (OLS) has been established to define the diagnosis of MCN-L. We present the case of a 33-year-old woman with a rapid progression of a cystic tumor of the liver. In 2 years, the lesion increased from 27 to 64 mm and a dilation of the left hepatic duct appeared. Percutaneous transhepatic drainage with a biopsy was performed. No malignant cells were found on biopsy. Because of the rapid progression of the cystic tumor and unclear malignant potential, left hemihepatectomy was performed. Even though tumor masses were present in the biliary duct, on the basis of the presence of OLS, histology finally confirmed MCN-L with intermediate-grade intraepithelial dysplasia to high-grade intraepithelial dysplasia. The patient is currently under oncologic follow-up with no signs of recurrence of the disease. We present a rare case where MCN-L caused a dilation of the left hepatic duct, a sign that is usually a characteristic of IPMN-B.     

Pathological classification of intrahepatic cholangiocarcinoma based on a new concept

Intrahepatic cholangiocarcinoma (ICC) arises from the lining epithelium and peribiliary glands of the intrahepatic biliary tree and shows variable cholangiocytic differentiation. To date, ICC was largely classified into adenocarcinoma and rare variants. Herein, we propose to subclassify the former, based on recent progress in the study of ICC including the gross classification and hepatic progenitor/stem cells and on the pathological similarities between biliary and pancreatic neoplasms. That is, ICC is classifiable into the conventional (bile duct) type, the bile ductular type, the intraductal neoplasm type and rare variants. The conventional type is further divided into the small duct type (peripheral type) and large bile duct type (perihilar type). The former is a tubular or micropapillary adenocarcinoma while the latter involves the intrahepatic large bile duct. Bile ductular type resembles proliferated bile ductules and shows a replacing growth of the hepatic parenchyma. Hepatic progenitor cell or stem cell phenotypes such as neural cell adhesion molecule expression are frequently expressed in the bile ductular type. Intraductal type includes papillary and tubular neoplasms of the bile duct (IPNBs and ITNBs) and a superficial spreading type. IPNB and ITNB show a spectrum from a preneoplastic borderline lesion to carcinoma and may have pancreatic counterparts. At invasive sites, IPNB is associated with the conventional bile duct ICC and mucinous carcinoma. Biliary mucinous cystic neoplasm with ovarian-like stroma in its wall is different from IPNB, particularly IPNB showing cystic dilatation of the affected ducts. Rare variants of ICC include squamous/adenosquamous cell carcinoma, mucinous/signet ring cell carcinoma, clear cell type, undifferentiated type, neuroendocrine carcinoma and so on. This classification of ICC may open up a new field of research of ICC and contribute to the clinical approach to ICC.     

Mucin-producing bile duct carcinoma originating at the hepatic hilus

We describe a mucin-producing bile duct carcinoma that was resected and analyzed pathologically. Endoscopic retrograde cholangiography disclosed amorphous filling defects in the dilated extrahepatic bile duct, suggesting that a large amount of mucin was excreted from the tumor. An intraductal amorphous filling defect showed shape change at different examination times. Pathology examination revealed a papillary tumor which showed mucus production, in the right hepatic duct. The cut section of the resected specimen showed a protuberant papillary lesion, measuring 14×14mm in diameter, forming a cystic mucous lake within the duct wall. Many cancer cells were also found in the mucous lake, the contents of which were strongly positive for mucin stain. The mucous lake developed laterally and communicated with the peribiliary glands, suggesting that the tumor had originated in these glands.     

Intraductal mucinous tumors occurring simultaneously in the liver and pancreas

A case of simultaneous intraductal mucinous tumors of the liver and pancreas in a 67-year-old man is described. Abdominal ultrasonography and computed tomography (CT) revealed the presence of cystic lesions with intraluminal septae both in the caudate lobe of the liver and in the uncinate process of the pancreas; these cystic lesions communicated with the hepatic duct and pancreatic duct, respectively. Mucin retention was observed in the cysts, and cholestasis was induced by mucin secretion into the common bile duct. The lesions were resected by left hepatic lobectomy with caudate lobectomy, and segmental pancreatectomy. Both lesions were multilocular cystic tumors with no papillary projections or focal mass effect in their walls. Histologically, both cystic lesions were a mixture of hyperplasia and adenoma lined by low papillary columnar epithelium. There were no cellular or histological features to suggest malignant change. The fibrous intratumor interstitium lacked any mesenchymal or ovarian-like stroma. The hepatic lesion was considered to be of a similar nature to intraductal papillary mucinous tumor (IPMT) of the pancreas. However, the two lesions occurred simultaneously in the liver and pancreas. This case is of interest in regard to the diagnosis and management of mucinous hepatopancreatobiliary lesions. Received: March 16, 2001 / Accepted: September 14, 2001     

Tumors with macroscopic bile duct thrombi in non-HCC patients: dynamic multi-phase MSCT findings

Non-hepatocellular carcinoma (non-HCC) with macroscopic bile duct tumor thrombus (BDTT) formation is rare, few radiological studies have been reported. In this case report, we retrospectively analyzed the imaging findings of three cases of non-HCC with macroscopic BDTT on dynamic enhanced multislice computed tomography (MSCT) scan. One case of primary hepatic carcinosarcoma was presented as a solitary, large well-defined tumor with significant necrotic changes. One case of liver metastasis from colon cancer was presented as a lobulated, large ill-defined tumor. One case of intraductal oncocytic papillary neoplasm involved the entire pancreas, presented as a cystic and solid mass with multilocular changes (the individual loculi were less than 5.0 mm in diameter). The bile duct was dilated due to expansible growth of the BDTT in all three patients. The BDTT was contiguous with hepatic or pancreatic tumor, and both of them showed the same enhancement patterns on dynamic contrast-enhanced computed tomography scan: early enhancement in the hepatic arterial phase and a quick wash-out of contrast agent in the portal and equilibrium phases. Macroscopic BDTT in non-HCC patient is rare, dynamic enhanced MSCT scan may be valuable in the diagnosis of non-HCC with BDTT.     

Intraductal Papillary Cholangiocarcinoma Associated With von Meyenberg Complexes: A Case Report

Intraductal papillary cholangiocarcinoma (IPC) is a rare form of bile duct neoplasm with only 28 cases reported in the English literature to date. We report a rare case of an IPC arising in a liver containing many von Meyenberg complexes (VMC) in a 70-year-old woman. She presented with colicky right upper quadrant pain and nausea, mimicking biliary colic. Imaging studies showed a mass in the left lobe of the liver. A left hepatectomy, which included segments 2 and 3 was performed, and an ill-defined shiny polypoid nonhomogenous mass (8.2×2.9 cm) was identified within the lumen of a dilated bile duct without invading the liver parenchyma. Histologically, the tumor was composed of papillary fronds with fine fibrovascular cores lined by tall biliary columnar mucus secreting epithelium. Focally the cells were of the gastric foveolar type. The tumor extended into the peribiliary glands and merged with an adjacent large VMC. A possibility that the carcinoma may have originated in a VMC was considered. On follow-up after 12 months the patient is doing well and is without any disease or recurrence.     

Intraductal papillary neoplasm of the bile duct developing in a patient with primary sclerosing cholangitis: A case report

We report a case of intraductal papillary neoplasm of the bile duct (IPNB) that developed in a patient with primary sclerosing cholangitis. A 46-year-old woman was admitted to our hospital with obstructive jaundice. The liver function tests demonstrated increased serum liver enzyme levels. Computed tomography showed dilatation of the intrahepatic bile ducts. Abdominal ultrasonography revealed a highly echoic protruding lesion in the posterior bile duct near the right lobe of the liver. The lesion was suspected to be IPNB, but we were unable to confirm whether it was a carcinoma. A right hepatectomy was performed, and this showed that the dilated bile duct was filled with mucin and contained several yellowish papillary tumors. Histologically, the neoplastic biliary epithelium showed papillary growth in the dilated lumen. The tumor was diagnosed as IPNB, high-grade intraepithelial neoplasia secreting abundant mucin. No recurrence has been detected 3 years after surgery.     

A case of a mucin-producing bile duct tumor diagnosed over the course of 6 years

We report a case of a mucin-producing intraductal papillary neoplasm of the intrahepatic bile duct (M-IPNB) diagnosed over a period of 6 years. A 64-year-old man underwent follow-up evaluations for an abdominal aortic aneurysm at our hospital. In 2009, a computed tomography (CT) scan revealed a simple hepatic cyst in segment 3 of the liver. Annual CT scans initially showed almost no change in the size or shape of the cyst. The cystic lesion, which measured 5 cm in 2014, had increased to 11 cm by 2015, and a solid component was detected within the cyst. A biliary cystic tumor was suspected and we performed a left lateral hepatectomy. Pathological examination showed that the papillary lesion in the cyst included adenocarcinoma and adenoma components. We diagnosed M-IPNB in 2015. Identification of the solid component of the cyst, as well as an increase in cyst diameter in the image analyses, was critical for diagnosis of M-IPNB.     

Clinicopathological features of "intraductal papillary neoplasm of the bile duct" and patient outcome after surgical resection

BACKGROUND/AIMS: Intraductal papillary neoplasm of the bile duct (IPNB) represents a biliary papillary tumor mainly growing in the bile duct lumen resembling intraductal papillary mucin-producing neoplasm of the pancreas. However, its clinical spectrum and characteristics have not been fully evaluated. METHODOLOGY: To define the clinicopathologic characteristics and prognosis of IPNB patients, 6 cases of IPNB who underwent surgical resection are presented. RESULTS: Patients were 3 males and 3 females, aged between 47 and 79 years. Five patients had histories of hepatobiliary disease. Imagery showed cystic or diffuse dilatation of the bile ducts. Tumor markers were not valuable for diagnosis. All patients underwent hemihepatectomy with or without resection of the caudate lobe or extrahepatic bile duct. Examination showed polypoid tumors in 5 cases though 1 case had no evident tumor. Mucin was observed in 3 cases. Five cases were well-differentiated adenocarcinoma and 1 had poorly differentiated adenocarcinoma. Vascular invasion was rare and lymph node metastasis was not observed. In-situ spread of carcinoma was seen along biliary mucosa in 3 cases. Five cases survived without tumor relapse for long periods but 1 died of tumor recurrence at 31 months. CONCLUSIONS: Complete resection of IPNB based on accurate preoperative assessment of tumor extension provides a good prognosis.     

Intraductal papillary neoplasm originating from an anomalous bile duct

An 82-year-old woman who had been suffering from repeated obstructive jaundice for 7 years was referred to our hospital. Although endoscopic aspiration of the mucin in the common bile duct had been temporally effective, origin of the mucin production had not been detectable. The patient thus had been forced to be on long-term follow-up without curative resection. Endoscopic retrograde cholangioscopy on admission revealed massive mucin in the common bile duct. In addition, an anomalous bile duct located proximal to the gallbladder was identified. Since the lumen of the anomalous duct was irregular and the rest of biliary tree was completely free of suspicious lesions, the anomalous duct was judged to be the primary site. Surgical resection of the segment 4 and 5 of the liver combined with the extrahepatic biliary tract was performed. Pathological diagnosis was compatible to intraductal papillary neoplasm with high-grade intraepithelial dysplasia of the anomalous bile duct. The patient has been free from the disease for 6.5 years after resection. This is the first case of intraductal papillary neoplasm derived from an anomalous bile duct, which was resected after long-term conservative treatment. The present case suggested the slow growing character of natural history of the neoplasm.     

Double cancer of the cystic duct and gallbladder associated with low junction of the cystic duct

We report a case of double cancer of the cystic duct and gallbladder associated with low junction of the cystic duct. A 73-year-old woman was admitted to the hospital complaining of upper abdominal pain. Endoscopic retrograde cholangiography showed a stenotic lesion in the lower common bile duct and no visualization of the cystic duct or gallbladder. Enhanced computed tomography revealed a heterogeneously enhanced tumorous lesion around the lower bile duct in the pancreatic head. A diagnosis of cancer arising from the cystic duct that entered the lower part of the common hepatic duct was made by intraductal ultrasonography, which showed an intraluminal protruding lesion in the cystic duct. Isolated gallbladder cancer was also diagnosed, by abdominal computed tomography. She underwent pancreaticoduodenectomy with dissection of regional lymph nodes. Histological examination revealed moderately differentiated adenocarcinoma of the cystic duct and well-differentiated adenocarcinoma of the gallbladder. Double cancer of the cystic duct and gallbladder is extremely rare, and this case also suggests a relationship between a low junction of the cystic duct and neoplasm in the biliary tract.     

Small pancreatic carcinoma misdiagnosed as superficially spreading cholangiocarcinoma

We report a case of small pancreatic carcinoma misdiagnosed as superficially spreading cholangiocarcinoma using percutaneous transhepatic cholangioscopy (PTCS). The patient was a 72-year-old man admitted to a local hospital with obstructive jaundice. The patient underwent percutaneous transhepatic biliary drainage and PTCS. He was referred to our hospital with a diagnosis of superficially spreading cholangiocarcinoma. Cholangiography revealed a stenosis of the common bile duct, and also revealed some irregularities from the common hepatic duct to the left hepatic duct, suggesting a superficial spread of cancer. No pancreatic tumor was identified by endoscopic retrograde pancreatography or by enhanced computed tomography. Cholangioscopy disclosed an elevated tumor with torsional vessels and granular mucosal lesions, which were extended to the left hepatic duct. Repeated cholangioscopic biopsies of the bile duct mucosa revealed adenocarcinoma. The patient was diagnosed with superficially spreading cholangiocarcinoma extending to the left hepatic duct and the right anterior hepatic duct. Left trisectionectomy combined with pancreatoduodenectomy was performed. The cut surface of the resected specimen showed a pancreatic head tumor that was 8 mm in diameter. Histological findings of the resected specimen revealed adenocarcinoma arising from the pancreatic head with invasion in the common bile duct. Additionally, extensive inflammatory granulation tissue was observed along the surface of the bile duct, without any evidence of carcinoma. This case implies to us that the results of PTCS, even after repeated biopsies, should be interpreted with great caution.     

Usefulness of peroral cholangioscopy in preoperative diagnosis of intraductal papillary neoplasm of the bile duct

Surgical resection is considered the first-line treatment of intraductal papillary neoplasm of the bile duct (IPNB). Since some patients with IPNB have an unexpectedly superficially-spreading lesion, preoperative cholangioscopic diagnosis is very useful in determining the extent of resection. We report a patient with IPNB in whom preoperative peroral cholangioscopic evaluation was useful.     

Left hepatic trisegmentectomy for intraductal papillary cholangiocarcinoma: report of a case

We present a rare case of intraductal papillary cholangiocarcinoma in a 69 year-old man which was treated with left hepatic trisegmentectomy. The hepatic bile ducts were dilated by intraductal masses, which had extended into the intrahepatic bile ducts without involvement of the posterior inferior segmental duct (B6). The patient underwent left hepatic trisegmentectomy with hilar duct resection. The tumors in the posterior superior segmental duct (B7) were resected and biliary reconstruction was performed with a jejunal loop. Post-operative recovery was good, and the patient survived for 7 months after surgery.     

Precancerous lesions of the biliary tree

The neoplasms of the biliary tree include the carcinomas of the intra- and extrahepatic bile ducts, the gallbladder and the ampulla. Two types of precancerous lesions precede these adenocarcinomas: the flat and non-tumour forming type that is called biliary intraepithelial neoplasia, and the papillary and tumour-forming type that has been named intraductal papillary neoplasm of the bile duct. Rarely also biliary mucinous cystic neoplasm can give rise to invasive biliary adenocarcinomas. This review discusses the pathological, molecular, epidemiological, clinical and prognostic features of the precancerous biliary lesions, separated according to their origin in the bile ducts, the ampulla and the gall bladder.     

Common features between neoplastic and preneoplastic lesions of the biliary tract and the pancreas

the bile duct system and pancreas show many similarities due to their anatomical proximity and common embryological origin.Consequently,preneoplastic and neoplastic lesions of the bile duct and pancreas share analogies in terms of molecular,histological and pathophysiological features.Intraepithelial neoplasms are reported in biliary tract,as biliary intraepithelial neoplasm(BilIN),and in pancreas,as pancreatic intraepithelial neoplasm(PanIN).Both can evolve to invasive carcinomas,respectively cholangiocarcinoma(CCA)and pancreatic ductal adenocarcinoma(PDAC).Intraductal papillary neoplasms arise in biliary tract and pancreas.Intraductal papillary neoplasm of the biliary tract(IPNB)share common histologic and phenotypic features such as pancreatobiliary,gastric,intestinal and oncocytic types,and biological behavior with the pancreatic counterpart,the intraductal papillary mucinous neoplasm of the pancreas(IPMN).All these neoplastic lesions exhibit similar immunohistochemical phenotypes,suggesting a common carcinogenic process.Indeed,CCA and PDAC display similar clinic-pathological features as growth pattern,poor response to conventional chemotherapy and radiotherapy and,as a consequence,an unfavorable prognosis.The objective of this review is to discuss similarities and differences between the neoplastic lesions of the pancreas and biliary tract with potential implications on a common origin from similar stem/progenitor cells.     

Intraductal papillary neoplasm of the bile duct accompanying biliary mixed adenoneuroendocrine carcinoma

We present the first case of an intraductal papillary neoplasm of the bile duct(IPNB) accompanying a mixed adenoneuroendocrine carcinoma(MANEC).A 74-yearold woman presented with fever of unknown cause.Laboratory data revealed jaundice and liver injury.Contrast-enhanced computed tomography revealed a 20 mm polypoid tumor in the dilated distal bile duct,which exhibited early enhancement and papillary growth.Upper gastrointestinal endoscopy revealed mucus production from the papilla of Vater,characterized by its protruding and dilated orifice.Endoscopic ultrasonography visualized the polypoid tumor in the distal bile duct,but no invasive region was suggested by diagnostic imaging.Therefore,the initial diagnosis was IPNB.After endoscopic nasobiliary drainage,a pylorus-preserving pancreaticoduodenectomy was performed.Pathological examination of the resected bile duct revealed papillary proliferation of biliary-type cells with nuclear atypia,indicating pancreaticobiliary-type IPNB.In addition,solid portions comprised of tumor cells with characteristic salt-and-pepper nuclei were evident.Immunohistochemistry revealed expression of the neuroendocrine marker synaptophysin in this solid component,diagnosing it as a neuroendocrine tumor(NET).Furthermore,the MIB-1 proliferation index of NET was higher than that of IPNB,and microinvasion of the NET component was found,indicating neuroendocrine carcinoma(NET G3).This unique case of MANEC,comprising IPNB and NET,provides insight into the pathogenesis of biliary NET.     

Multicystic biliary hamartoma with extremely elevated CA19-9: a case report

Multicystic biliary hamartoma (MCBH) is a rare cystic disease of the liver. We herein report a case of MCBH associated with extremely elevated levels of serum carbohydrate antigen (CA) 19-9. A 53-year-old man was referred to our hospital because of extremely elevated CA19-9 levels (more than 12,000?U/mL). Enhanced abdominal computed tomography and magnetic resonance imaging (MRI) revealed a multicystic tumor with a calcified wall in the left lobe of the liver, although no apparent intracystic nodule was detected. Because of the possibility of a malignant tumor, such as intraductal papillary neoplasm of the bile duct or cystadenocarcinoma, the patient underwent left hepatectomy. Based on the postoperative pathological findings, the lesion was diagnosed as MCBH. The serum CA19-9 level drastically decreased after surgery. We encountered a rare case of MCBH with extremely elevated CA19-9 levels.