ABSTRACT
The mucin-hypersecreting biliary papillomatosis is a premalignant neoplasm characterized by intraductal papillary proliferation involving extensive areas of the intrahepatic and/or extrahepatic bile duct. We report a case of mucin-hypersecreting biliary papillomatosis manifested as obstructive jaundice and diagnosed only by microscopy, with a review of literatures. A 74-year-old female, who had a past history of cholecystectomy about 13 years ago, was admitted to our hospital with jaundice. A CT scan showed marked dilatation of intrahepatic and extrahepatic bile duct without intraductal filling defect or extrabiliary mass. During endoscopic retrograde cholangiopancreatography, mucin extrusion from the duodenal major papilla and dilated common bile duct with amorphous filling defects was noted. Percutaneous transhepatic biliary drainage for cholangioscopy was failed. In the operation field, there was a lot of mucin but was no visible mass at the common bile duct with bare eyes and cholangioscopy. However, papilloma was detected at the random biopsy specimen by microscopy. The patient underwent partial resection of common bile duct and choledocho-jejunal anastomosis.
Subject(s)
Aged , Female , Humans , Bile Duct Neoplasms/diagnosis , Cholangiopancreatography, Magnetic Resonance , Mucins/metabolism , Papilloma/diagnosis , Tomography, X-Ray ComputedABSTRACT
Biliary papillomatosis (BP) is a rare disease that is classified as either the mucin-hypersecreting type or nonmucin-secreting type. Typical papillomas of the biliary tree mainly involve intrahepatic bile ducts or both intra- and extrahepatic bile ducts. The isolated involvement of the extrahepatic bile duct is rare, and there is no report of BP confined to the gallbladder in Korea. We report a case of a 50-year-old man who developed obstructive jaundice due to hypersecreted mucin from papillomas of the gallbladder.
Subject(s)
Humans , Middle Aged , Bile Ducts, Extrahepatic , Bile Ducts, Intrahepatic , Biliary Tract , Carcinoma, Papillary , Gallbladder , Jaundice, Obstructive , Korea , Mucins , Papilloma , Rare DiseasesABSTRACT
BACKGROUND/AIMS: Mucin-hypersecreting bile duct tumor is rare, and has an unusual histologic characteristic of having various degrees of cellular atypia ranging from dysplasia to invasive carcinoma in the same specimen. To gain insight into the role of p16, p14 and p53 in the carcinogenic process of bile duct tumor, we analyzed the expression status of these proteins in mucin-hypersecreting bile duct tumor. METHODS: Immunohistochemical staining of p16, p14 and p53 were performed in 34 paraffin embedded tissues obtained from 22 patients of mucin-hypersecreting bile duct tumor. RESULTS: Thirty-four specimens were categorized into low-grade dysplasia (9), high-grade dysplasia (4), carcinoma in situ (CIS, 11) and invasive carcinoma (10) based on the degree of cytologic and structural atypia. p53 overexpressions were found in 6 (17.6%, 3 in CIS, 3 in invasive carcinoma) and more frequently observed in the advanced histologic stages (p<0.05). Loss of p16 staining was found only in 2 (6%) of low-grade dysplasia specimen. Loss of p14 staining was found in 21 (61.7%, 7 in low-grade dysplasia, 2 in high-grade dysplasia, 8 in CIS, and 4 in invasive carcinoma) and was frequently observed in low-grade and high-grade dysplasia compared to p53 (p<0.05). CONCLUSIONS: In mucin-hypersecreting bile duct tumor, p14 and p53 may play a role in the early and advanced stage of carcinogenesis, respectively. Further study regarding genetic and epigenetic alterations in p14 and p53 gene may be needed.
Subject(s)
Adult , Aged , Female , Humans , Male , Middle Aged , Bile Duct Neoplasms/genetics , Carcinoma/genetics , Cyclin-Dependent Kinase Inhibitor p16/genetics , English Abstract , Genes, p16 , Genes, p53 , Immunohistochemistry , Mucins/metabolism , Mutation , Tumor Suppressor Protein p14ARF/geneticsABSTRACT
The mucin-hypersecreting bile duct tumor is rare and its clinical, radiologic, and pathologic features are not well known. We report the case of mucin-hypersecreting biliary papillomatosis with malignant transformation including review of 11 korean literatures. A 65-year-old female was admitted to our hospital with fever, chills, and right upper quadrant pain. A CT scan showed marked dilatation of left intrahepatic duct with intraductal filling defects. During endoscopic retrograde cholangiograpy, mucin from the ampulla was observed and dilated common bile duct with multiple amorphous filling defects was noted. Cholangioscopy revealed muliple coral-like mucosal papillary projections with large amount of mucin in the left intrahepatic ducts. The patient underwent left lobectomy, and the biopsy revealed intraductal papillary adenocarcinoma in the background of papillary adenoma.
Subject(s)
Aged , Female , Humans , Adenocarcinoma , Adenocarcinoma, Papillary , Adenoma , Bile Ducts , Biopsy , Chills , Common Bile Duct , Dilatation , Fever , Mucins , Papilloma , Tomography, X-Ray ComputedABSTRACT
BACKGROUND/AIMS: Mucin-hypersecreting cholangiocarcinoma is very similar to intraductal papillary mucinous tumor (IPMT) of the pancreas. But this disease is rare and its clinicopathologic features are not well known. The purposes of this study are to report our experience of eight patients with the tumor and to analyze the characteristics of its clinical, radiological and pathological finding. METHODS: Eight patients (3 men and 5 women, mean age 52 years) were diagnosed as having a mucin-hypersecreting cholangiocarcinoma among 4,570 cases of ERCP from 1995 to 1998. RESULTS: Recent or previous attacks of biliary pain or acute cholangitis were elicited in most of the patients. CT showed diffuse intra- and extra-hepatic duct dilatation in all patients and suspicious ill-defined mass shadow in 2 out of 8 patients. On cholangioscopic examination, a papillary mass or minute mucosal lesion was found in the dilated bile duct containing thick viscid mucin. Eight patients were recommended an operation and curative resection was performed in seven patients. Histologically, well-differentiated adenocarcinoma in the background of benign hyperplasia and adenoma was documented in all patients. CONCLUSIONS: Mucin- hypersecreting cholangiocarcinoma may be characterized by exceedingly similarity to IMPT of the pancreas in clinical, radiological and pathological features. Further investigation is needed for detailed information such as natural history and outcome.
Subject(s)
Female , Humans , Male , Adenocarcinoma , Adenoma , Bile Ducts , Cholangiocarcinoma , Cholangiopancreatography, Endoscopic Retrograde , Cholangitis , Dilatation , Hyperplasia , Mucins , Natural History , PancreasABSTRACT
Intraductal papillary mucinous tumor (IPMT) of the pancreas is a recently understood unique clinicopathologic disease entity comprising approximately 1% of all exocrine pancreatic tumors and 11% of cystic neoplasms of pancreas. It has been reported worldwide, mostly in Japan. It is generally characterized by recurrent pancreatitis, mucin oozing from the papilla of Vater, and dilated pancreatic duct with intraductal filling defects. Microscopically, the mucin-producing columnar epithelium forms papillary proliferation into the dilated pancreatic duct and this feature differentiates IPMT of the pancreas from the more common mucinous cystic neoplasms of the pancreas which usually do not communicate with the pancreatic duct. On the other hand, mucin-hypersecreting bile duct tumors have been rarely reported in the English literature. We herein present the first case of mucin-hypersecreting bile duct tumor combied with IPMT of the pancreas with a review of the related literature.