Surgical treatment of mucin-producing cholangiocarcinoma arising from intraductal papillary neoplasm of the intrahepatic bile duct: a report of 2 cases

Intraductal papillary neoplasms of the bile duct (IPNB) leads to malignant transformation and mucin production. Herein, we presented two cases of mucin-producing IPNB with obstructive jaundice who underwent resection of the intrahepatic lesions and bypass hepaticojejunostomy. The first case was a 69 year-old male patient with 5-year follow up for gallstone disease. Imaging studies showed mucin-secreting IPNB mainly in the hepatic segment III bile duct (B3) and multiple intrahepatic duct stones for which, segment III resection, intrahepatic stone removal, end-to-side choledochojejunostomy and B3 hepaticojejunostomy were conducted. The second case was a 74 year-old female patient with 11-year follow up for gallstone disease. Imaging studies showed mucin-producing IPNB with dilatation of the segment IV duct (B4) and mural nodules for which, segment IV resection, partial resection of the diaphragm and central hepaticojejunostomy were conducted. Both patients recovered uneventfully from surgery. These cases highlight that in patients with IPNB, abundant production of highly viscous mucin inducing obstructive jaundice may be associated with malignant transformation.

Peroral Direct Cholangioscopic-Guided Biopsy and Photodynamic Therapy Using an Ultraslim Upper Endoscope for Recurrent Hepatocellular Carcinoma with Intraductal Tiny Nodular Tumor Growth

Bile-duct invasion is rare in patients with hepatocellular carcinoma (HCC). We report a case that received peroral direct cholangioscopy (PDCS)-guided endoscopic biopsy and photodynamic treatment (PDT) for recurrent HCC with intraductal tiny nodular tumor growth. A 64-year-old woman presented with recurrent right upper-quadrant pain. Six months previously she had been diagnosed with HCC with bile-duct invasion in the right anterior segment and had received right anterior segmentectomy. On pathological examination, the margin of resection was clear, but macroscopic bile-duct invasion was noted. On admission, magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography (ERCP) revealed a 0.5-cm-sized polypoid mass at the hilar portion. ERCP-guided biopsy failed, and an ampullary stricture was noted. PDCS-guided endoscopic biopsy was thus performed, and histopathology of the retrieved specimen revealed HCC. The patient submitted to PDT. There was no procedure-related complication. After 1 month of PDT the polypoid lesion and scar change at the hilar lesion had disappeared.

Clinical Features of Intrahepatic Cholangiocarcinoma according to Gross Morphology

PURPOSE: Recently, the Liver Cancer Study Group of Japan classified intrahepatic cholangiocarcinoma into three types: mass forming type, periductal infiltrating type and intraductal growth type. The clinical features of these three types are not well known. The purpose of this study was to define the clinical features of intrahepatic cholangiocarcinoma according to gross morphology. METHODS: We retrospectively reviewed the clinical records of 98 patients with intrahepatic cholangiocarcinoma who had undergone surgery at the Department of Surgery, Seoul National University Hospital from January 1980 to December 1998. The tumors were classified into mass forming type (MF, n=42), periductal infiltrating type (PI, n=22), intraductal growth type (IG, n=21) and Mixed type (n=13) by gross appearance. RESULTS: There were no differences in age, sex ratio, symptoms or laboratory findings. Intrahepatic stones were highly associated with the PI type (31.8% vs 2.4% in MF p=0.02). Hepatitis B surface antigen was more frequently found in the MF type (21.4% vs 4.5% in PI, 4.8% in IG, p=0.04). The size of tumor in the MF type was larger than those of the PI and IG types. In PI type, the rate of lymph node metastasis was higher (45.5% vs 19% in MF, 0% in IG, p=0.01). In IG type, the tumors were associated with adenomatous hyperplasia at a 95% rate. The cumulative five year survival rate of the MF and IG types were 23.3% and 76.2% (p<0.001), respectively. There were no five year survivors in the PI type. CONCLUSION: Intrahepatic cholangiocarcinoma has quite different clinical features and prognoses according to the grosstypes. Therefore we must choose appropriate treatment strategies according to gross type.