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Hepatobiliary cystadenoma is a rare hepatic neoplasm which has a high tendency to recur and has malignant potential. We review the literature and report a case of intrahepatic cystadenoma presenting with abdominal pain and ascites following spontaneous rupture. Complete excision of the tumor offers the best chance of cure and avoids recurrence, secondary infection or malignant change.

OBJECTIVE: The aim of this investigation was to describe the clinical features, diagnosis, pathologic characteristics, and optimal surgical management for patients with extrahepatic biliary cystadenomas. SUMMARY BACKGROUND DATA: Extrahepatic biliary cystadenomas are rare epithelial neoplasms. The clinical features and optimal surgical management for these lesions have not been defined clearly. The usual presenting symptom is jaundice. These lesions should be considered premalignant and necessitate resection. Sporadic case studies have reported instances of recurrence with local excision. To the authors' knowledge, this study represents the largest collected single series of extrahepatic biliary cystadenomas and reviews previously reported cases. METHODS: The authors reviewed and reported their institutional experience from 1950 to 1993 in treating seven patients with extrahepatic biliary cystadenomas as well as 19 previously reported cases in the literature. RESULTS: A strong female predominance (96.3% of patients reviewed) was associated with extrahepatic biliary cystadenomas. Obstructive jaundice was the most common presenting symptom (85%). Abdominal pain occurred in 50% of patients; other symptoms included fever and hemobilia. The most common site of occurrence was the common hepatic duct (32%). Papillary cystadenoma with foci of invasive adenocarcinoma, thus supporting the malignant potential of cystadenomas, occurred in one patient. Local excision from the wall of the bile duct was performed in 18 patients and was associated with 50% recurrence within a mean follow-up of 13 months (range, 4-24 months). No recurrence was reported after formal sleeve resection and bilioenteric reconstruction. CONCLUSIONS: Extrahepatic biliary cystadenomas can become malignant, and in this study, local surgical excision was associated with a 50% local recurrence rate. Sleeve resection with negative histologic resection margins followed by bilioenteric reconstruction, therefore, is recommended.

Hepatobiliary cystadenomas are rare, truly benign cystic epithelial tumors with the potential to become cystadenocarcinoma. The initial symptom is vague, nonspecific abdominal pain, frequently in middle-aged women, but patients may be asymptomatic. Abdominal ultrasonography may be diagnostic. Intracystic fluid, obtained by sonographically guided fine-needle aspiration, may contain elevated levels of carcinoembryonic antigen. Complete resection or enucleation is the treatment of choice. We present the cases of two patients with hepatobiliary cystadenoma.

We report on a case of 61 year old female who presented with upper abdominal discomfort. Ultrasound and computerized tomography scan revealed a liver cyst resembling a hydatid cyst. Signs of mild biliary obstruction were present in laboratory tests. At operation, a cystadenoma of the left liver protruding into the common bile duct was found. Left hepatectomy with resection of the common bile duct and right side cholangiojejunostomy were performed.

Cholangiomas (bile duct adenomas) are seen mainly in old dogs and cats. They have also been reported in swine, rats, birds 2,7 and mice. Reports of adenomas of the gall bladder are rare, occurring mainly in cattle. There are individual reports of cholangiomas in a reindeer and a green iguana, as well as several reports in white perch. To our knowledge, this tumor has not been described in the goat. The purpose of this report is to present the gross and histopathologic findings on a cholangioma found in a goat during slaughter inspection. The liver was submitted to the pathology section of the

Like in adenomas of the colon, an adenoma-carcinoma sequence is expected from adenomatous growths in the papilla of Vater. Papilloadenomas must therefore be resected. Imaging techniques have a decisive influence on the choice of operative technique and consequently on perioperative mortality. Using conventional endoscopic ultrasonography, detection of lesions smaller than 10 mm in diameter is rare. The probes available up to now for intraductal sonography with an external diameter of between 3.4 and 12 mm were too rigid and could be inserted transpapillarily in only a few cases. The recent development, however, of high-frequency, extremely flexible ultrasound catheters with an external diameter of 1.17 mm makes intraductal examination of the pancreatic ductal system possible. We present a case of a 66 year-old female patient, in whom, for the first time, a histologically adenomatous lesion of the papilla of Vater could be examined with intraductal sonography using the endoscopic retrograde, transpapillary approach. As there was no evidence of infiltrative growth, the patient underwent a modified duodenum-preserving resection of the pancreatic head. Histological examination of the resected specimen confirmed the preoperative intraductal sonography findings. Since conventional endosonography did not detect the lesion in this case, intraductal sonography with flexible, high-frequency catheters as presented here, clearly may have a role in extending the possibilities of preoperative examination, especially of focal lesions in the periampullary region.

A 59-year-old male with history of sigmoid colon cancer had a high serum-CEA level and was referred for the evaluation of metastatic liver disease. Ultrasonography and computerized tomography showed two tumours in the liver. Macroscopically, these were in segment 4 (S4) and 2 (S2). Histologically, the tumour in S4 showed a number of bile ductules with variable amounts of stroma, an appearance compatible with bile duct adenoma (BDA). There were markedly atypical ductules of various sizes, the epithelium of which had coarsely granular/hyperchromatic large nuclei, in some areas of the lesion. These atypical ductules showed invasive growth into the liver parenchyma. Some cystically dilated ductules with bile plugs resembling bile duct hamartoma (BDH) were also seen. The other tumour in S2, was a metastatic adenocarcinoma from sigmoid colon and showed strongly positive staining for CEA. Since the lesion in S4 of our case is solitary and most of histological features are similar to those of BDA with markedly atypical bile ductules, we consider that this may be the first case of cholangiocarcinoma associated with BDA with focal area of BDH. It is possible that the adenoma-carcinoma sequence occurs in biliary tumours.

Patients with liver tumors are known to reveal antioxidant system disorders which lead to accumulation of products of lipids peroxidation and lower resistance. Levels of malonic dialdehyde as well as the antioxidant system (superoxide dismutase, catalase, alpha-tocopherol and retinol) in liver and tumor have been followed in 28 patients in whom liver was removed to treat malignant tumors. Liver and tumor tissue were shown to contain more dialdehyde and less superoxide dismutase and catalase than in the livers of accident victims. Treatment with alpha-tocoferol (600 mg), retinol (100,000 MU) and ascorbic acid (1.5 g) for 7 days before surgery was found to significantly reduce dialdehyde level in the liver. Also, the catalase level increased. Treatment with alpha-tocoferol and retinol resulted in their selective accumulation in the liver. No changes in lipid peroxidation or accumulation of alpha-tocoferol in tumor were recorded. Purulent and septic complications were 1.6 times less frequent after preoperative antioxidant treatment than in controls. It is recommended that said antioxidant treatment should be used to correct lipid peroxidation and to improve the effectiveness of therapy of liver cancer.

PURPOSE This prospective, nonrandomized trial evaluated a percutaneous isolated chemotherapy perfusion approach for treating advanced primary and metastatic liver tumors. Chemotherapy was administered via hepatic artery catheter and hepatic venous blood isolated by a novel percutaneous double-balloon inferior vena cava (IVC) catheter was passed through a detoxification/filtration cartridge in a venovenous bypass circuit. PATIENTS AND METHODS Among 23 patients enrolled onto the study, 58 procedures were performed on 21 patients. Twelve patients received dose escalations of fluorouracil (5-FU) (1,000 mg/m2 to 5,000 mg/m2), and nine received dose escalations of doxorubicin (50 mg/m2 to 120 mg/m2). Pharmacokinetic studies included drug accumulation in the liver, extraction by detoxification filters, systemic exposure, and alterations of half-life. Each patient received two treatments at 3-week intervals. Those showing stabilization or response received additional treatments. RESULTS There was a direct relationship between dose and peak concentration of drug entering the hepatic veins. The system functioned efficiently throughout the dose range, with extraction efficiencies ranging from 64% to 91% (P < .001). The hepatic vein drug levels showed a sixfold increase in 5-FU with dose escalation from 1,000 to 5,000 mg/m2, and a twofold increase in dox with dose escalation from 50 to 120 mg/m2 (P < .001, filter-mediated drug extraction). The treatments were accomplished with only an overnight hospital stay and no mortality. The common procedure-related toxicity was transient hypotension (grade I to II), due to catecholamine depletion by the filter. Dose-limiting toxicity (leukopenia) was observed in patients receiving 5-FU at a dose of 5,000 mg/m2 and doxorubicin at a dose of 120 mg/m2. Significant tumor response (> 95% reduction) was obtained in two patients receiving doxorubicin at 90 mg/m2 and 120 mg/m2. CONCLUSION The use of a double-balloon catheter to isolate and detoxify hepatic venous blood during intraarterial therapy is technically feasible, safe, and allows administration of large doses of intrahepatic chemotherapy at short intervals. This approach should allow new dose-intensification strategies to increase tumor responses in primary and metastatic liver tumors.

Vinyl chloride is a DNA-damaging carcinogen which induces liver angiosarcomas in humans and animals. Activation of the Ki-ras 2 gene by a GC--AT transition at the second base of codon 13 in human liver angiosarcomas associated with occupational exposure to vinyl chloride has been reported recently. In order to compare the molecular pathways of carcinogenesis in humans and animals, Sprague-Dawley rats were exposed to vinyl chloride and hepatic tumors, including two hepatocellular carcinomas and five liver angiosarcomas, were investigated for mutations at codons 12, 13 and 61 of the Ha-ras, Ki-ras and N-ras genes. High molecular weight DNA was amplified by the polymerase chain reaction and point mutations were analyzed by allele specific oligonucleotide hybridization, direct sequencing of polymerase chain reaction products and sequencing after cloning. None of the tumors exhibited a mutation in codons 12, 13 and 61 of the Ki-ras gene, nor in codons 12 of the Ha-ras gene or 61 of the N-ras gene. However, an activating AT--TA transversion at base 2 of codon 61 of the Ha-ras gene was detected in the two hepatocellular carcinomas. Mutations involving codon 13 (GGC--GAC) and codon 36 (ATA--CTA) of the N-ras A gene were detected in two liver angiosarcomas, suggesting that the nature of the ras gene affected by a given carcinogen depends on host factors specific to cell types. Several additional base pair substitutions were found in exon 1 of the N-ras B and C sequences. NIH 3T3 transfection assays and Southern blot analysis of DNA from transformed NIH 3T3 cells confirmed the presence of a dominant activated N-ras gene. These results emphasize the differences in the molecular pathways leading to tumors in humans and rats and within a given species between different cell types.

The medical and necropsy records of 41 cats diagnosed with nonlymphomatous hepatobiliary (NLHB) masses, including neoplasia and cysts, were reviewed. Overall, benign masses (n = 27) were more common than malignant ones (n = 14). The single most common malignancy was cholangiocellular carcinoma. The median age at diagnosis was significantly lower (P < .01) for cats with malignant rather than benign disease. Clinical signs associated with hepatobiliary neoplasia were usually vague and included lethargy, vomiting, and anorexia, often present for at least 2 weeks before presentation. Benign masses were an incidental finding in significantly more (P < .01) of the cases than were malignant masses. Median values for alanine aminotransferase (ALT), aspartate aminotransferase (AST), and total bilirubin were significantly higher (P < .05) in cats with malignant versus benign masses. The prognosis for malignant disease was poor, with 86% of the cats dying or being euthanatized during hospitalization. Cats with benign disease that underwent exploratory celiotomy were more likely to recover and warranted a more favorable prognosis than cats with malignant tumors. Factors associated with malignancy included age at presentation, presence of clinical signs at presentation, and specific serum chemistry changes.

The case of a 12-year-old boy with the diagnosis of intrahepatic biliary duct cystadenoma is presented. A critical review of the literature revealed less than 100 cases described at present. Differential diagnosis with other intrahepatic cystic lesions of varied etiology is discussed too.

The authors report the case of a 37-year-old woman with a biliary cystadenoma that mimicked a liver cyst. The magnetic resonance (MR) imaging features of this rare lesion were correlated with the pathologic findings, showing the potential of MR imaging for depicting and aiding in the diagnosis of biliary cystadenoma and its recurrence.

Silver-binding nucleolar organizer regions (AgNORs) were counted in sections from formalin-fixed, paraffin-embedded tissue blocks of 14 benign and 17 malignant tumors of minor salivary glands. The difference in the mean number of AgNORs count per nucleus between benign and malignant tumors was found to be statistically significant (p0.001). A positive correlation between the AgNORs count and the degree of malignancy of the malignant salivary gland tumors was generally observed. The AgNORs technique can be used as a diagnostic aid in differentiating between benign and malignant salivary gland tumors, and possibly the salivary duct carcinoma from the acinic cell carcinoma and poorly differentiated mucoepidermoid carcinoma.

A series of 107 patients with cholangiocarcinoma diagnosed between January 1980 and December 1991 is reported. Changing patterns of investigation and treatment in the periods 1980–1985 and 1986–1991 are analysed. There was a decrease in the use of percutaneous transhepatic cholangiography in the second period (86 versus 51 per cent of patients) but increased use of endoscopic retrograde cholangiography (19 versus 71 per cent) and computed tomography (8 versus 59 per cent). The overall resectability rate (17 per cent) was similar to those of other reported series but greater in the second period (8 versus 21 per cent). Palliation by endoscopic and percutaneous stenting was associated with a high incidence of recurrent cholangitis (55 per cent) and jaundice (35 per cent). During the second 6-year period, more effective palliation was achieved by segment III cholangiojejunostomy with a lower incidence of recurrent cholangitis (19 per cent) and jaundice (19 per cent). Overall prognosis for patients with this condition is grim and efforts must usually be aimed at providing the most appropriate palliation.

Hepatic cyst fluid cytology tends to yield disappointing results. We report a case of a 56-year-old woman with a biliary cystadenocarcinoma diagnosed by fine needle aspiration cytology. Computed tomography scans had shown a solitary, unilocular hepatic cyst over a five-year period. There was a recent increase in the size and development of a mural echogenic focus. Cytologic examination revealed clusters of malignant glandular cells in a background of cellular debris and mucinophages. The resected specimen confirmed the presence of an adenocarcinoma arising from malignant transformation of a preexisting cystadenoma.

Biliary cystadenoma is a rare benign neoplasm usually found in the parenchyma of the right lobe of the liver. The lesions are usually septated and represent less than 5 percent of non-parasitic cysts of biliary origin. Malignant degeneration to biliary cystadenocarcinoma is well documented, and recurrence is the rule following incomplete excision.

Hepatobiliary cystadenoma is a rare hepatic lesion characterized by a multiloculated cyst lined by cuboidal or columnar epithelial cells. Four cases of hepatobiliary cystadenoma with mesenchymal stroma (HCMS) and one case of hepatobiliary cystadenoma with intracystic epithelial component were studied by light microscopy, immunohistochemical methods, and electron microscopy. Similar studies were conducted on six fetal gallbladder tissues, representing the biliary tree, and two adult ovarian tissues. By light microscopy, the columnar epithelium of the five cases of hepatobiliary cystadenoma was similar to the epithelium of the developing gallbladder. The spindle cell stroma of the HCMS and the subepithelial spindle cells of the developing gallbladders showed similar reactivity to smooth-muscle actin. Vimentin reactivity was strongly positive in the stroma of the HCMS, and in the fetal gallbladders it was only noted in the subepithelial spindle cells of the 15-week gestation fetal gallbladder tissues. By electron microscopy, the epithelium lining the hepatic lesions showed characteristic gastrointestinal features and was identical to the epithelia lining the embryonic gallbladders. Furthermore, the mesenchymal stroma of the HCMS recapitulated the features found in subepithelial tissues in developing gallbladders. Although the ovarian stroma resembled the stroma of the HCMS by light microscopy, the immunohistochemical reactions and the electron microscopic studies showed dissimilarities. This study supports the hypothesis that the hepatobiliary cystadenomas arise from ectopic embryonic tissues destined to form the adult gallbladder.

Background. Although there have been a few reports dealing with the sarcomatous changes of intrahepatic cholangiocarcinoma, its clinicopathologic features as well as immunohistochemical nature remain obscure. Methods. Among 155 cases of intrahepatic cholangiocarcinoma, 7 cases of sarcomatous cholangiocarcinoma were chosen. Immunohistochemical studies using the avidin-biotin-peroxidase complex method were performed on these cases. Results. The tumor showed both mucin-producing adenocarcinoma areas and sarcomatous areas, the latter being predominant in three cases and focal in the other four. All the sarcomatous areas consisted of atypical spindle cells arranged in sheets or bundles. Pleomorphic giant cells were observed in some sarcomatous components in five cases. Immunohistochemical staining for keratin and epithelial membrane antigen revealed apparent positivity in the sarcomatous components of five cases. The patients with these tumors showed aggressive intrahepatic spreading and widespread metastasis of the sarcomatous cells, and demonstrated poorer prognosis than those with ordinary cholangiocarcinoma, with one exception, a patient who remained disease-free for 3 years after surgery. Conclusions. These findings favor the possible epithelial origin of sarcomatous cells. Radical operation would be necessary for patients with this special type of cholangiocarcinoma.

Operative ultrasonography for orienting the direction of transection of the liver is often useful in obtaining an adequate disease-free surgical margin. We have devised a new technique for hepatectomy guided by needles inserted under ultrasonographic guidance.One hundred two hepatectomies were performed between January 1987 and September 1991, and the hepatectomy with this technique was begun in January 1989.In 10 of 29 limited hepatectomies performed in the first phase of the period in which this technique was not available, disease-free surgical margin of less than 1 cm was left because of inadequately directed division. Disease-free surgical margin of more than 1 cm was left in 18 of 23 limited hepatectomies in the second phase of the period in which this technique was available. In the other five operations where disease-free surgical margin of less than 1 cm was left, carcinomas were located too close to the major hepatic vessels. The average blood loss during the limited hepatectomies was reduced by this technique. Two-year and 3-year survival of patients undergoing hepatectomy for hepatocellular carcinoma were more favorable in the second phase than in the first phase.Although the difference between the two groups was not significant, this technique is useful in performing adequate transection of the liver.

Primary sclerosing cholangitis (PSC) predisposes to the development of cholangiocarcinoma, a usually fatal complication that is difficult to diagnose. Serum concentrations of CA 19-9, a tumor-associated antigen, are frequently increased in patients with only cholangiocarcinoma. The aim of this study was to assess the value of an increased serum CA 19-9 level for the diagnosis of cholangiocarcinoma in patients with preexisting PSC. We analyzed serum samples from 9 patients with PSC and superimposed cholangiocarcinoma and from 28 patients with only PSC. Serum concentrations of CA 19-9 were measured in a blinded manner with use of an immunoradiometric assay. The serum CA 19-9 concentrations were increased in 8 of 9 patients (89%) with PSC and cholangiocarcinoma (mean +/- SE, 391 +/- 86 U/ml; range, 4 to 677), whereas they were increased in only 4 of 28 patients (14%) with only PSC (mean +/- SE, 61 +/- 16 U/ml; range, 2 to 370). The sensitivity of a CA 19-9 value greater than 100 U/ml for cholangiocarcinoma in PSC was 89%, and the specificity was 86%. The measurement of serum concentrations of CA 19-9 is a promising test for detecting cholangiocarcinoma in patients with PSC.

Although the association of cholangiocarcinoma with intrahepatic calculi (hepatolithiasis) is well recognized, the late development of cholangiocarcinoma after the treatment of hepatolithiasis has not been reported in detail. Of 109 consecutive patients with hepatolithiasis treated during 19 years, eight patients had cholangiocarcinoma, seven of whom had cholangiocarcinoma two to 14 years, with a mean of eight years, after the treatment of hepatolithiasis. Absence of cholangiocarcinoma was confirmed when stones were removed at the time of the initial treatment. The mean age was 56 years, with a female to male ratio of 2:5. At the time of detecting cholangiocarcinoma, three patients had no gallstones and four had gallstones at the corresponding site to the carcinoma. Cystic dilatation of the intrahepatic bile duct was often observed on the direct cholangiogram. The biles were all infected mainly with Escherichia coli and Klebsiella species. Thus, bile stasis and bacteria infection seems to be the important causative factor, causing cholangiocarcinoma rather than the calculi itself. Because the symptoms only mimic those of cholangitis, the possible presence of cholangiocarcinoma should be considered even after the treatment of hepatolithiasis for early detection and curative resection.

Our purpose was to identify the conventional spin-echo and contrast-enhanced dynamic MR imaging features of intrahepatic cholangiocarcinoma.Eight patients with pathologically proved intrahepatic cholangiocarcinoma were examined with conventional spin-echo and contrast-enhanced dynamic MR imaging with a 1.5-T superconductive unit. The results were analyzed to include the signal intensity of the mass relative to liver parenchyma on conventional spin-echo images and the pattern and degree of enhancement on dynamic MR images.The characteristic appearance of intrahepatic cholangiocarcinoma was a large mass with an irregular margin, satellite nodules, and a central scar. Tumors were hypointense relative to liver parenchyma on T1-weighted spin-echo images and hyperintense on T2-weighted spin-echo images. On dynamic MR studies, tumors characteristically had minimal or moderate rim enhancement with progressive and concentric filling with contrast material.Our study suggests that intrahepatic cholangiocarcinoma has a typical pattern on contrast-enhanced dynamic MR images.

The role of intraduodenal bile in regulation of plasma cholecystokinin (CCK) levels were investigated in patients with obstructive jaundice under external bile diversion and under physiological bile flow into the duodenum by internal bile drainage. Basal plasma CCK levels determined by a specific and sensitive bioassay in patients under external bile drainage (2.2 +/- 0.2 pmol/liter; mean +/- SE) were significantly higher than those in control subjects (1.0 +/- 0.3 pmol/liter). In control subjects, the peak CCK response (6.2 +/- 0.7 pmol/liter) to a test meal was seen at 45 min, whereas that in patients under external bile drainage, it was seen at 20 min after a test meal (17.6 +/- 3.2 pmol/liter; P0.01 vs controls). After peak response, plasma CCK levels in controls gradually decreased, but remained significantly elevated during a 3-hr observation period. In patients under bile diversion, the test meal caused a prompt plasma CCK peak, with a transient fall followed by a continuous rise until 180 min postprandially. In six patients, external bile diversion was changed to internal biliary drainage with a stent tube within two weeks to maintain physiological bile flow into the duodenum. Internal bile drainage normalized basal (0.9 +/- 0.2 pmol/liter) as well as meal-stimulated CCK release (peak value: 5.0 +/- 0.8 pmol/liter). These results demonstrate that endogenous bile exerts tonic inhibition on basal and postprandial plasma CCK levels in humans.

Expression of pancreatic lipase in normal, proliferating, and carcinomatous epithelia of the intrahepatic biliary tree was examined by immunohistochemistry in 82 normal livers, 35 hepatolithiatic livers, 11 cholangiocarcinomas (CCs) associated with hepatolithiasis, 34 CCs, and four combined hepatocellular-cholangiocellular carcinomas. The intrahepatic biliary tree was anatomically divided into large ducts, septal ducts, interlobular ducts, bile ductules, and peribiliary glands. In hepatolithiasis, large ducts, septal ducts, and peribiliary glands showed marked proliferation and dysplasia. In normal livers and hepatolithiasis, expression of pancreatic lipase was found in large ducts in 91% and 94%, in septal ducts in 95% and 94%, and in peribiliary glands in 93% and 94%, respectively. Interlobular ducts, bile ductules, and hepatocytes were negative for pancreatic lipase. The immunoreactivity of pancreatic lipase was coarse granular, and was regularly present in the supranuclear and to a lesser degree paranuclear cytoplasm of the epithelial cells. All cases of CCs with hepatolithiasis, which arised from large ducts, expressed pancreatic lipase. In CCs, pancreatic lipase was expressed in the perinuclear cytoplasm of cancer cells in 67% in the hilar type and in 24% in the peripheral type (P0.02). The combined hepatocellular-cholangiocellular carcinomas failed to express pancreatic lipase in both elements. These data suggest that large ducts, septal ducts, and peribiliary glands contain pancreatic lipase in normal and proliferative conditions, and that CCs probably arising from these ductal elements continue to express pancreatic lipase. Thus, pancreatic lipase could be a phenotypic marker of large ducts, septal ducts and peribiliary glands as well as their malignant counterparts.

First documented case of biliary cystadenoma in a non-white elderly female is described, showing (so called) low-density daughter cysts within the mother cyst emphasising the fact that presence of daughter cysts within a mother cyst cannot be considered pathognomonic of hepatic hydatid cyst.

Diagnostic and therapeutic findings of benign hepatic tumors are analysed. In particularly the authors describe the more frequent tumors such as angioma, adenoma and FNH, but also give a guidelines on how to approach less frequent lesions.

It is reported that a prompt increase in the arterial ketone body ratio (acetoacetate/beta-hydroxybutyrate), which reflects the mitochondrial redox state of the liver graft, is a decisive prerequisite for graft survival in clinical liver transplantation. To contrast the rôle of hepatic mitochondrial redox state in partial hepatectomy with that in hepatic replacement, the changes in the ketone body ratio were investigated in 107 cases of hepatectomy. The ketone body ratio in uneventful cases (n = 70) in the first three days after hepatectomy was significantly higher than that in eventful cases. In the uneventful cases, the ketone body ratios were all increased to above 1.0 within two days after hepatectomy, except in diabetics, whose preoperative values did not reach 1.0 under oral glucose load. However, 20 (22.7%) out of 88 cases whose ketone body ratios promptly increased after hepatectomy had mild to moderate complications thereafter. It is suggested that the recovery of hepatic mitochondrial redox state is also a prerequisite in partial hepatectomy, where a reduced and often damaged liver confronts systemic metabolic load.

A review of seven hepatic resections in six patients undergoing maintenance hemodialysis is presented. These cases consisted of hepatocellular carcinoma in four, and cholangiocellular carcinoma, myelolipoma and focal nodular hyperplasia in one each. The last preoperative hemodialysis was undertaken within 24 h prior to the operation with heparin. Intraoperatively, infused solutions containing no potassium, along with strict attention to preventing overhydration, allowed us to manage the patients without hemodialysis on the day of the operation. No specific intra-operative complications related to hemodialysis were noted. Postoperative hemodialysis was performed on the first or second day after operation, using nafamstat mesilate, a synthetic protease-inhibiting agent. The morbidity rate in the hemodialyzed patients was 85.7% (6/7), which was significantly higher than that in the non-hemodialyzed patients who underwent hepatic resections in our hospital. Fluid collection in the pleural and/or peritoneal cavities was frequent and difficult to control, but transient. Our experience suggests that hepatic resection is an acceptable procedure for hemodialyzed patients, when used in conjunction with careful perioperative management.

Recent progress has been made in identifying patients with malignant primary liver tumors who have the best chance of long-term survival after orthotopic liver transplantation. Patients with UICC stages I or II hepatocellular carcinoma, the fibrolamellar variant of hepatocellular carcinoma, or epithelioid hepatic hemangioendothelioma are acceptable candidates for orthotopic liver transplantation. Carefully selected patients with neuroendocrine tumors metastatic to the liver may also have long-term palliation with liver transplantation. Therapeutic strategies are currently being developed to minimize disease recurrence after liver transplantation for patients with advanced-stage hepatocellular carcinoma confined to the liver or with cholangiocarcinoma. However, published survival rates for most patients with malignant primary liver tumors remain suboptimal, and significant progress in preventing disease recurrence is required before liver transplantation can be widely applied to most patients with primary malignant liver tumors.

The authors present a case of Caroli's disease complicated with multiple lithiasis and cholangiocarcinoma. They analyse the etiopathogenesis and the anatomopathologic features of this uncommon disease and express some diagnostic and therapeutic considerations.

Distinguishing primary hepatocellular carcinoma (HCC) from metastatic carcinomas to the liver is often difficult, if not impossible, particularly in needle biopsy and fine-needle aspiration specimens. In an attempt to identify a specific immunohistochemical profile that would distinguish HCC from metastatic carcinomas, we studied 56 HCCs, 8 cholangiocarcinomas, and 24 metastatic adenocarcinomas with monoclonal antibodies to alpha-fetoprotein (AFP), keratin (AE1, AE3, and CAM5.2), Leu-M1, human milk fat globule (HMFG-2), tumor-associated glycoprotein-72(B72.3), epithelial specific membrane antigen (Ber-EP4), and BCA-225 (CU-18). Both monoclonal and polyclonal (mCEA and pCEA) antibodies to carcinoembryonic antigen also were used. Metastatic adenocarcinomas were often positive for CU-18(71%), Leu-M1 (75%), B72.3 (50%), HMFG-2 (67%), Ber-EP4(83%) and mCEA(71%). Using these antibodies, the frequency of positivity for HCC was 9%, 16%, 11%, 20%, 36%, and 11%, respectively. CU-18 was the only monoclonal antibody in which there was a significant difference in positive rates between HCC and metastatic adenocarcinomas. Most HCCs (71%) revealed a bile canalicular staining pattern with pCEA. Because this staining pattern was absent in metastatic carcinomas, pCEA appears to be useful in confirming a diagnosis of HCC. AE1, AE3 and CAM5.2 antibodies were not useful in distinguishing HCC from metastatic carcinomas. Each cholangiocarcinoma shared a staining profile similar to that of metastatic carcinomas.

This study analyzed new cell lineage markers for the differential diagnosis between hepatocellular carcinoma (HCC) and cholangiocarcinoma (ChC), as well as the potential pathways of cell-cell and cell-extracellular matrix interactions of neoplastic liver cells during tumor spread and invasion, by comparing the expression of (VLA) integrins, vitronectin receptor, and neural cell adhesion molecule in normal, inflamed, and neoplastic human liver biopsies. All cases of liver cell adenoma and well-differentiated HCC expressed the same set of integrins as observed in normal liver tissue, i.e., VLA-alpha 1 and VLA-beta 1. Poorly differentiated HCC also expressed VLA-alpha 1 and VLA-beta 1, but in addition de-novo expressed VLA-alpha 2, VLA-alpha 3, VLA-alpha 6 and vitronectin receptor. All cases of well-differentiated ChC expressed an identical integrin immunoprofile as observed in normal bile duct epithelium, i.e., VLA-alpha 2, VLA-alpha 3, VLA-alpha 6, VLA-beta 4 and vitronectin receptor, whereas poorly differentiated ChC showed a markedly decreased expression of these integrin subunits. VLA-alpha 1 was constantly absent from all cases of ChC, whereas VLA-beta 4 was never expressed by HCC. Neural cell adhesion molecule, exclusively expressed by proliferating reactive bile ductules in cholestatic and regenerating liver, was constantly absent from both malignant neoplasms. In conclusion, the integrin make up of various liver tumors closely follows that of their normal counterparts. Differences in integrin receptor expression vary according to the cellular origin of the tumors and are associated with a poor differentiation. Our findings suggest that immunohistochemical staining for VLA-alpha 1 and VLA-beta 4 integrin subunits, which highlight the cellular phenotype of the two neoplasms, might be a helpful tool in the differential diagnosis between HCC and ChC.

Hepatolithiasis associated with cholangiocarcinoma is not often encountered. During the past 3 years, an increased incidence of patients with hepatolithiasis associated with cholangiocarcinoma was noted. Data were needed to reliably determine the incidence of this disease.Data concerning the relationship between cholangiocarcinoma and hepatolithiasis are presented. The treatment modalities and factors that influence long-term survival are discussed.The overall incidence of cholangiocarcinoma in association with hepatolithiasis was 5.0% (55 in 1105). Before 1987, 65% of cholangiocarcinoma in association with hepatolithiasis was diagnosed postoperatively. After 1987, the incidence of accurate preoperative diagnosis increased (22.8%), and in most of the other instances (62.8%), the diagnosis was made at laparotomy. Surgical procedures consisted of common bile duct exploration with T-tube drainage (100%) and hepatectomy (38.2%). Mortality for patients who underwent surgery was 5.4%; they died of recurrent cholangitis. The overall median survival time of patients with cholangiocarcinoma in association with hepatolithiasis was 10.4 months; the 1-, 2-, and 4-year cumulative survival rates were 30.0%, 12.7%, and 3.6%, respectively. Patients with hepatectomy or the presence of mucobilia had better survival rates (P0.05).The overall incidence of hepatolithiasis associated with cholangiocarcinoma was 5%. In most patients with cholangiocarcinoma in association with hepatolithiasis, diagnosis can be made preoperatively and at laparotomy. Patients with hepatectomy or presence of mucobilia had better survival rates.

An interportal communicating branch was found at the hepatic bifurcation in two (2.4%) out of 83 hepatectomized cases of carcinoma of the biliary tract. The first case was a 54-year-old male with intrahepatic bile duct cancer, and the second was a 64-year-old female with gallbladder cancer. No other anatomical anomalies were found in the portal vein, hepatic artery, or biliary tract.

Tumor necrosis interfered with conventional methods of stenting in a patient with hilar cholangiocarcinoma. Therefore, a hepaticoduodenal fistula was percutaneously catheterized and dilated, and a large caliber endoprosthesis inserted to drain the right hepatic ductal system.

Endoscopic stent placement has become accepted palliative therapy for malignant biliary tract obstruction. Because stent occlusion remains a significant late complication, prophylactic replacement has been suggested, although the appropriate time interval remains unclear. Patients with malignant biliary strictures who received 10F or 11.5F stents were analyzed with respect to clinical response, occlusion rates at 3 and 6 months, and survival rates. Seventy stents were placed in 50 patients. Pancreatic carcinoma was the most common underlying malignancy. Overall, obstructive symptoms resolved in 94% of cases. Occlusion rates at 3 months (4.2%) and 6 months (10.8%) were not significantly different. Median overall survival averaged 22 weeks. Results were also stratified by underlying diagnosis, with the worst clinical response and survival being seen in the group of patients with metastatic cancer. Findings suggest that the time interval for stent replacement can be extended safely from 3 to 6 months, resulting in decreased patient discomfort and cost and obviating any replacement in that significant percentage of patients who expire before 6 months.

The case of a 71-year-old man with thorotrastosis and a cholangiocarcinoma detected by MRI is presented, and the radiographic appearance of the cholangiocarcinoma is discussed. We concluded that MR is more useful for detecting Thorotrast-induced liver tumors than US and CT, but CT is more useful for the detection of Thorotrast deposits.

In this retrospective study, the authors compared the sensitivity and specificity, in terms of the diagnosis of malignancy, of two techniques of ultrasound-guided biopsy of liver tumours. The first technique involving fine needle biopsy with cytological and histological analysis of the specimen was carried out in 39 patients. A technique using a wide calibre needle with frozen section examination of the fragment obtained was performed in 27 patients. All patients had one or more malignant liver tumours. Sensitivity and specificity of the technique with frozen section histology were both 100 per cent, while figures for the technique with delayed histological study were 84.6 and 87.4 per cent. This study suggests that frozen section histology might be associated with greater sensitivity than delayed histology.