Mucin-producing Bile Duct Tumor Research Articles

Clinicopathological features and prognosis of mucin-producing bile duct tumor and mucinous cystic tumor of the liver: A multi-institutional study by Japan biliary association

<h3>Background</h3> The aim of this study was to determine the clinicopathological features and surgical outcomes of mucinous cystic neoplasm of the liver (MCN) and mucinproducing intraductal papillary neoplasm of the intrahepatic bile duct (M-IPNB). <h3>Methods</h3> We performed a multi-institutional, retrospective study of patients with MCN or M-IPNB pathologically defined by the presence or absence of an ovarian-like stroma. <h3>Results</h3> The M-IPNB and MCN were diagnosed in 119 and nine patients, respectively. MCN was observed in female patients, while M-IPNB produced symptoms of cholangitis. M-IPNBs were classed as low or intermediate grade in 53 cases, high grade in 23 and invasive carcinoma in 43. Fifty-one of the M-IPNBs were the pancreatobiliary type (PT), 33 were the intestinal type (IT), 23 were the oncocytic type (OT), and 12 were the gastric type (GT). The 1-, 5- and 10-year survival rates for the 105 patients with M-IPNB were 96%, 84% and 81%, respectively, while the 5-year survival rate for patients with MCN was 100%. OT and GT M-IPNB had better 10-year survival rates than PT and IT M-IPNB. <h3>Conclusions</h3> Although MCN has different features from M-IPNB, both diseases have a good prognosis after resection.

A case of mucin producing bile duct tumor

A case of mucin producing bile duct tumor

A Mucin-producing Bile Duct Tumor with Multilocular Cystic Growth and Varied Histopathological Findings

多彩な病理組織像を示した粘液産生性胆管腫瘍の1例を経験したので報告する．症例は78歳の男性で，1日前からの下腹部痛，腹満症状にて当院受診となった．CT，超音波検査で虫垂炎と肝S4に内部隆起を有する30mm大の嚢胞性腫瘤を認め，入院となった．MRI，造影超音波検査ではmultilocularな嚢胞性腫瘤を認め，内部隆起性病変に一部造影効果を認めた．DIC-CTでは左肝管に狭小化を認めたが，内部に粘液は認めなかった．肝嚢胞性腫瘍の診断で左葉切除を施行した．病理組織学的検査所見ではさまざまな程度に乳頭状増殖した粘液産生性腫瘍上皮からなる多嚢胞性病変で，胆管との交通を認めた．腫瘍上皮は，carcinomaからadenomaまで多彩な異型を呈した．粘液形質は，MUC1，MUC5AC，MUC6は一部陽性で，MUC2は陰性だった．分枝型膵管内乳頭粘液性腫瘍に類似した粘液産生性胆管腫瘍と考えられた．

Significance of mucin expression in pancreatobiliary neoplasms

Mucins are high molecular weight glycoproteins that play important roles in carcinogenesis and tumor invasion. We have described, for the first time, that pancreatic ductal adenocarcinomas (PDACs) with an aggressive behavior and a poor outcome expressed MUC1 (pan-epithelial membrane-associated mucin) but did not express MUC2 (intestinal-type secreted mucin), whereas intraductal papillary mucinous neoplasms (IPMNs) with indolent behavior and a favorable outcome did not express MUC1 but did express MUC2. These expression profiles of MUC1 and MUC2 related to the prognoses of the patients were also observed in biliary neoplasms such as intrahepatic cholangiocarcinoma (ICC)-mass-forming type (MF), mucin-producing bile duct tumor (MPBT), and extrahepatic bile duct carcinoma (EHBDC). We also found recently that high expression of MUC4 (tracheobronchial membrane-associated mucin) in PDACs, ICCs-MF, and EHBDCs was a new independent poor prognostic factor, although MUC4 was not expressed in normal pancreatobiliary tissue. High de novo expression of MUC5AC (gastric-type secreted mucin) was observed in many types of pancreatobiliary neoplasms, including all grades of pancreatic intraepithelial neoplasia (PanIN) and biliary intraepithelial neoplasia (BilIN), and all types of IPMNs and MPBTs, as well as PDACs and ICCs-MF, although MUC5AC was not expressed in normal pancreatobiliary tissue. The combined status of MUC1, MUC2, MUC4, and MUC5AC expression may be useful for the early detection of pancreatobiliary neoplasms and evaluation of their malignancy. In regard to the mechanism of mucin expression, we have recently reported that MUC1, MUC2, MUC4, and MUC5AC gene expression is regulated by epigenetics (DNA methylation and histone H3 lysine 9 modification) in cancer cell lines, including PDAC cells. Translational research of mucin gene expression mechanisms, including epigenetics, in pancreatobiliary neoplasms may give us new tools for the early and accurate detection of these neoplasms.

Tumorigenesis and phenotypic characteristics of mucin‐producing bile duct tumors: an immunohistochemical approach

Intraductal papillary neoplasm of the bile duct (IPNB) is characterized by exophytic proliferation of neoplastic epithelial cells with fibrovascular stalks in bile duct lumen, mucin hypersecretion, and considerable dilatation or multilocular changes of the affected bile ducts. A mucin-producing bile duct tumor is an IPNB with excessive mucin production and clinical symptoms. Herein, the phenotypes as well as the tumorigenesis and progression of IPNB are reviewed with immunohistochemical assistance. The tumors are subdivided into three phenotypes: pancreatobiliary, intestinal, and gastric. About half of IPNB cases are of the pancreatobiliary type, and the remaining half are of the intestinal type. Aberrant expression of CDX2 with MUC2 and CK20 is related to the development of intestinal metaplasia. Inactivation of P16INK4a and nuclear expression of beta-catenin are related to the development of IPNB. Decreased expression of membranous beta-catenin and E-cadherin and aberrant expression of MMP-7 and -9 and of MUC1 are related to invasion of IPNB with tubular adenocarcinoma, whereas MUC2 is involved in the invasion of IPNB with mucinous carcinoma. IPNB can be regarded as a counterpart of intraductal papillary mucinous neoplasm (IPMN) of the pancreas, particularly the main duct type. More comparative studies between IPNB and pancreatic IPMN are recommended for further analysis of these papillary neoplasms.

Cholangioscopy by Using Narrow-Band Imaging and Transpapillary Radiotherapy for Mucin-Producing Bile Duct Tumor

Cholangioscopy by Using Narrow-Band Imaging and Transpapillary Radiotherapy for Mucin-Producing Bile Duct Tumor

A case of mucin producing bile duct tumor resembling intraductal papillary mucinous neoplasm of the pancreas

A case of mucin producing bile duct tumor resembling intraductal papillary mucinous neoplasm of the pancreas

Mucin-producing Bile Duct Tumor of the Caudate Lobe Protruding into the Common Hepatic Duct

Mucin-producing tumor in the bile duct is referred to clinically as mucin-producing bile duct tumor (MPBT). Intraductal papillary neoplasm of the biliary tract that resembles an intraductal papillary mucinous neoplasm (IPMN) of the pancreas is a rare category of MPBT and is not well characterized. We, herein, report a case of MPBT of the caudate lobe of the liver that showed papillary growth and communicated with the bile duct of the caudate lobe and protruded into the common hepatic duct. Histologically, MPBT cells showed papillary overgrowth with abundant mucinous secretions, resembling an IPMN of the pancreas. The MPBT cells showed the same immunostaining pattern as that of cells from IPMN of the pancreas.

Aberrant expression of pyloric gland‐type mucin in mucin‐producing bile duct carcinomas: A clear difference between the core peptide and the carbohydrate moiety

The authors have recently defined the clinopathological entity of a mucin-producing bile duct tumor (MPBT), and divided MPBT into two distinct subtypes: 'columnar-type' and 'cuboidal-type' MPBT. Mucin core protein 6 (MUC6), which is present in normal pyloric glands, had higher expression levels in cuboidal-type tumors than in columnar-type tumors. In the pyloric glands, a carbohydrate antigen detected by monoclonal antibody HIK1083 (CA/HIK1083) is also expressed. In order to evaluate the coexpression pattern of MUC6 and CA/HIK1083 in MPBT, expression profiles were evaluated in 38 surgically excised mucin-producing bile duct carcinomas (MPBC; cuboidal-type, n = 15; columnar-type, n = 23), using immunohistochemistry. The staining rate was graded as follows: -, <5% of neoplastic cells stained; +, 5% to <20%; + +, 20% to <50%; + + +, > or =50%. In cuboidal-type MPBC, MUC6 was positive in all cases (+ + +, 13/15; + +, 1/15; +, 1/15), whereas CA/HIK1083 was negative in all cases (-, 15/15; P < 0.0001). In columnar-type MPBC, MUC6 was positive in 65% of cases (+ + +, 6/23; + +, 8/23; +, 1/23; -, 8/23), and CA/HIK1083 was positive in 52% (+ +, 3/23; +, 9/23; -, 11/23; not significant). Our results clearly demonstrate that cuboidal-type MPBC have an aberrant pyloric glandular phenotype, that is, MUC6+/CA/HIK1083-. This unique profile may be related to different outcomes of patients with MPBC.

A CASE OF MUCIN PRODUCING BILE DUCT ADENOMA WITH REPEATED ACUTE PANCREATITIS

Mucin producing bile duct tumor is a rare disease and 104cases of the disease have been described in the Japanese literature. Of those 104 cases, 91cases were malignant and only 7 cases included ours were benign. We experienced a case of mucin producing bile duct adenoma with repeated acute pancreatitis. A 48-year-old woman was admitted to the hospital for with a diagnosis of repeated acute pancreatitis. ERCP revealed a defect in a dilated common bile duct and a slightly dilated posterior intrahepatic bile duct. EST was performed and revealed the defect in the common bile duct was mucin. No abnomality was found in the pancreatic duct. Peroral cholangioscopy revealed that mucin was localised only in a posterior intrahepatic bile duct. Posterior hepatic segmentectomy was performed under the diagnosis of mucin producing bile duct tumor of posterior intrahepatic bile duct. The resected specimen showed a flat glanular elevation lesion with the diameter of 7mm in locally dome like dilated posterior intrahepatic bile duct. The histopathological diagnosis was papillary adenoma.

A case of mucin-producing bile duct tumor which responded to bromhexine hydrochloride treatment and radiotherapy

A case of mucin-producing bile duct tumor which responded to bromhexine hydrochloride treatment and radiotherapy