Nonbacterial thrombotic endocarditis and cerebral embolism in a patient with cholangiocarcinoma.

We present a patient who has cholangiocarcinoma, nonbacterial thrombotic endocarditis (NBTE) and cerebral embolic infarction. This is the first case report of NBTE proved by pathology in Taiwan.

Endosonography in the clinical staging of Klatskin tumor.

Endosonography was performed preoperatively in 46 patients with carcinoma of the common hepatic duct and its bifurcation. The results of endosonography were correlated with findings during surgery and pathological examination of the resected specimen and classified according to the new (1987) TNM classification. Overall accuracy in assessing the depth of tumor infiltration was 86.0%. Endosonography was accurate in predicting the presence of lymph nodes but not accurate in defining non-metastatic changes of lymph nodes. Staging of distant metastases was not accurate due to the low penetration depth of ultrasound.

Hepatic resection using a water jet dissector.

The mortality and morbidity in major hepatic resection is often related to hemorrhage. A high pressure, high velocity water jet has been developed and has been utilized to assist in hepatic parenchymal transection. Sixty-seven major hepatic resections were performed for solid hepatic tumors. The tissue fracture technique was used in 51 patients (76%), and the water jet dissector was used predominantly in 16 patients (24%). The extent of hepatic resection using each technique was similar. The results showed no difference in operative duration (p = .499). The mean estimated blood loss using the water jet was 1386 ml, and tissue fracture technique 2450 ml (p = .217). Transfusion requirements were less in the water jet group (mean 2.0 units) compared to the tissue fracture group (mean 5.2 units); (p = .023). Results obtained with the new water dissector are encouraging. The preliminary results suggest that blood loss may be diminished.

Malignant biliary obstruction: efficacy of thin-section dynamic CT in determining resectability.

OBJECTIVE: Several authorities advocate the use of preoperative angiography to determine the resectability of pancreatic and periampullary tumors, claiming that CT alone is not sufficiently accurate for this purpose. Our objective was to assess the value of CT in predicting surgical resectability in patients with malignant biliary obstruction. MATERIALS AND METHODS: We performed a retrospective analysis of 380 consecutive cases of malignant biliary obstruction spanning a 4-year period. Most patients (230) were treated nonoperatively. Sixty-seven patients had surgery, pathologic confirmation of malignancy, and preoperative CT scans available for review. The CT scans were assessed for surgical resectability of tumor by an interpreter who did not know the patient's history. RESULTS: Forty-two patients had pancreatic adenocarcinoma, six had ampullary carcinoma, seven had cholangiocarcinoma, and 12 had other malignant neoplasms. Of 47 patients with tumors thought to be unresectable on the basis of CT findings, 42 had tumors that were found to be unresectable at surgery (positive predictive value, 89%). Of 20 patients with tumors thought to be resectable, 16 had tumors that were surgically resectable (positive predictive value, 80%). CT did not show metastases to duodenal lymph nodes (n = 2), portal vein infiltration (n = 1), and small hepatic metastases (n = 1). Visualization of most of these at angiography would not be expected. The CT finding of infiltration of the periarterial fat around the celiac or superior mesenteric arteries was reliable for predicting surgical unresectability. Lymphadenopathy and infiltration of nonperivascular fat planes were less reliable predictors of unresectability. CONCLUSION: Although some findings on CT that suggest unresectability are less reliable than others, the accuracy of CT compares favorably with reports on the accuracy of angiography for assessing tumor resectability in cases of malignant biliary obstruction. The addition of angiography to the examination of patients with potentially resectable lesions is not justified when high-quality, thin-section dynamic CT has been performed.

[An autopsy case of pulmonary metastasis of cholangiocellular carcinoma associated with marked fibrotic change of the lungs].

An autopsy case of pulmonary metastasis of cholangiocellular carcinoma is presented. A 44-year-old woman was admitted to our hospital because of dyspnea, general fatigue and a sense of abdominal fullness on February 5, 1990. In November 1986, at an other hospital, she had been diagnosed as having diffuse metastatic lung tumor and multiple bone metastases, by transbronchial lung biopsy and other examinations. During the clinical course, she was not received chest irradiation and chemotherapy which induced fibrotic change of lungs. Chest X-ray film on December 21, 1986 showed diffuse nodular shadows in both lung fields. Chest X-ray film on February 4, 1990 showed diffuse reticular shadows with marked shrinkage of lung fields. She died two months after admission. The primary site of the carcinoma was not determined clinically, but was revealed by autopsy to be cholangiocellular carcinoma of the liver, with generalized metastasis. Microscopic findings of the autopsied lung showed markedly increased connective tissue around bronchi and blood vessels, in areas where microtubular adenocarcinoma was scattered. This is a very rare case of pulmonary metastasis of cholangiocellular carcinoma, associated with marked fibrotic change of the lungs during about 3.5 years. To our knowledge, this is the first reported case.

MRI manifestations of peritoneal carcinomatosis.

Three cases of proved peritoneal carcinomatosis were examined by magnetic resonance imaging (MRI). Air was used to distend the entire gastrointestinal tract via an antegrade method. The findings included seedings along the small intestine, transverse and sigmoid colon, stellate pattern in the mesentery, plaque-like and bulky tumor masses in the mesentery and greater omentum, and focal thickenings along the right subdiaphragmatic parietal peritoneum. Stenosis caused by tumor encasement at the duodenojejunal junction and ileocolic anastomosis were first detected by MRI and later confirmed by barium studies. Ascites was present in all cases. One case showed ascites located only along the left paracolic gutter. This report shows that MRI is also able to demonstrate peritoneal carcinomatosis by using air as a gastrointestinal contrast medium.

MR imaging of peripheral cholangiocarcinoma.

A prospective study was performed to compare MR spin-echo (SE) sequences [repetition time/echo time (TR/TE) 2,000/80, 500/44 ms], unenhanced CT, and rapid intravenous contrast enhanced CT in eight consecutive patients with peripheral cholangiocarcinoma. All the tumors (ranging from 5 to 9.6 cm in size) were detected with all four techniques. Tumor contrast, however, was qualitatively greatest on long TR/TE SE images. With long TR/TE SE images, tumors were demonstrated as well-demarcated homogeneous regions of high signal intensity, and the anatomic relations between tumors and intrahepatic blood vessels were easily perceived. Detection of small intrahepatic metastatic foci was best on long TR/TE images. Tumor invasion of the portal vein's branches was also best seen on long TR/TE SE images. These results indicate that long TR/TE SE sequence is the most effective initial screening method in demonstrating the presence and determining resectability of peripheral cholangiocarcinoma.

Hepatic transplantation for primary and metastatic cancers of the liver.

Long-term results of transplantation for primary and metastatic hepatic malignancies were evaluated retrospectively in 637 patients. Recurrence rates and 2-year and 5-year patient survival rates were calculated. The overall recurrence rate was 40%, with 81% of deaths from recurrence occurring within 2 years after transplantation. Best results were obtained with uncommon tumors: incidental hepatomas (13% recurrence; 57% 2-year and 5-year follow-up); epithelioid hemangioendotheliomas (33% recurrence; 82% and 43% 2-year and 5-year survival); hepatoblastomas (33% recurrence; 50% 2-year and 5-year survival); and fibrolamellar hepatomas (39% recurrence; 60% and 55% 2-year and 5-year survival). Hemangiosarcomas had 64% recurrence, and all patients died within 27.5 months. Tumors metastatic to the liver had 59% recurrence, with 38% and 21% 2-year and 5-year survival rates. Transplantation should be abandoned for hemangiosarcomas and most metastatic tumors, except possibly for some slowly growing neuroendocrine tumors. The usual hepatomas had 39% recurrence with 2-year and 5-year survival rates of 30% and 18%, respectively. Cholangiocarcinomas had 44% recurrences with 2-year and 5-year survival rates of 30% and 17%, respectively. Transplantation for hepatomas and cholangiocarcinomas should be reserved for patients with favorable risk factors or when combined with well-defined chemotherapy protocols before and after operation.

Primary liver cancer in non-cirrhotic liver. Epidemiological study based on autopsies performed in Trieste, Italy and Kurume, Japan.

This study, aimed at elucidating the epidemiological features of primary liver carcinoma developing in non-cirrhotic livers, was based on 25,103 autopsies performed between 1975 and 1984 in Trieste, Italy. These autopsies correspond to approximately 70% of all deaths that occurred in this area. Various factors allegedly related to carcinomas were analysed in reference to our previous study on cirrhotic livers and in comparison with 5,603 autopsies in Kurume, Japan. There were 28 cases of hepatocellular carcinoma (HCC), 16 of cholangiocellular carcinoma (CCC) not associated with cirrhosis in Trieste, and 48 HCC and 19 CCC in Kurume. On the basis of our findings, it was concluded that cirrhosis, regardless of its cause, is the main pathogenetic factor in HCC; it is responsible for a much higher frequency (14.2:1) than in non-cirrhotic livers, as well as for early occurrence of tumours (an average of 6 years earlier in cirrhotic liver) in Trieste. Patients in Trieste were older than those in Japan, and the frequency of HCC among all autopsies was much greater in the latter. By contrast, the influence of cirrhosis on cholangiocellular carcinoma (CCC) was negligible, as such association appeared purely coincidental or absent. The incidence of CCC among autopsies was greater in Japan. Our data on CCC were not sufficient to demonstrate any clear aetiopathogenetic association between this tumour and alcohol abuse and hepatitis B virus (HBV) infection, except for a possible aetiological role of gallstones. The frequency of CCC relative to HCC was greater in Trieste than in Japan; the incidence of HCC was much less in Trieste, whereas CCC was more frequent in Japan.

Primary hepatic malignancies: resection or liver transplantation?

Liver transplantation in malignancies must be confined to patients with potentially curable disease. The indication is widely accepted, however, in non-resectable tumors or in patients with cirrhosis that excludes major resection. Without treatment prognosis is extremely poor in these patients. In our own experience 12 out of 13 non-cirrhotic patients with hepatocellular carcinoma (HCC) died within 9 months, and 17 out of 19 cirrhotic patients died within the first year of non-curative or explorative surgery. None of our patients with HCC in non-cirrhotic livers has lived longer than 38 months, and those with cirrhotic livers more than 61 months even after curative resection. After liver transplantation 1-year survival rate was 54% in 14 patients with primary hepatic carcinomas (12 HCC, 2 CCC). In cirrhotic patients with large or infiltrating HCC the results of resection are worse than after grafting, at least in the Western World, so liver transplantation must be taken into consideration. The lack of grafts limits treatment by transplantation in these patients. Transplantation is only exceptionally indicated for patients with metastatic liver disease.

Acquired myasthenia gravis and cholangiocellular carcinoma in a dog.

Acquired myasthenia gravis and cholangiocellular carcinoma were diagnosed in a 7-year-old English Setter referred because of forelimb lameness, exercise-induced weakness, and fever. Three months earlier, the dog had had a pleuropulmonary infection caused by a Fusobacterium sp. The concurrent development of myasthenia gravis and cholangiocellular carcinoma in this dog may be explained by a paraneoplastic syndrome, although it is unproven. The cholangiocellular carcinoma may have possessed an acetylcholine receptor-like antigen on the tumor surface, which induced autoantibodies to cross-react with acetylcholine receptors at the neuromuscular junction.

Brain and skull metastases of hepatic or pancreatic cancer--report of six cases.

Brain and skull metastases from primary hepatic or pancreatic cancer are very rare. The authors describe six cases of metastatic tumors. These are skull (three cases) and brain (one) metastasis of hepatic cancer and brain metastasis (two) of pancreatic cancer. In three hepatic cancer patients, the metastatic lesions were diagnosed before the diagnosis of primary cancer. In these patients, plain skull x-ray showed osteolytic lesions and vascular enlargement. A postcontrast computed tomographic (CT) scan showed an enhanced high-density epidural mass. Angiograms showed a tumor stain fed by abnormal vessels from the external carotid artery. In one patient with a metastatic brain tumor from hepatic cancer, a CT scan showed a high-density mass with hematoma. In one of the brain metastases from pancreatic cancer, a CT scan revealed a cystic, ring-like enhanced lesion in the thalamus. In the other case, a CT scan showed an isodensity mass in the vermis and hydrocephalus. Metastatic tumors from primary hepatic cancer were soft and hemorrhagic, but they were clearly demarcated from the surrounding tissue. In the case of thalamic metastasis, the cyst content was aspirated and an anticancer agent was administered into the cystic cavity. In the other cases, the tumors were totally removed. The outcome was very poor in all cases.

Clinical significance of implantation metastases after surgical treatment of cholangiocarcinoma.

Cytologic examination of bile obtained during surgery from intrahepatic bile ducts in patients with malignant proximal bile duct obstruction has shown a high incidence of tumor cells. Spill of bile occurs frequently during these operations and postoperative bile leakage often occurs. Typical implantation metastases were detected in three patients who underwent resective surgery for bile duct cancer. In addition, peritoneal spread of bile duct carcinoma was found on postmortem examination in seven of ten patients who died 6 to 27 months after resection of the hilar tumor. A relation between tumor-positive bile cytologic findings, tumor spill, and seeding during surgery is likely to exist. It is recommended that during surgery the utmost care should be taken to prevent spill of bile.

The role of radiotherapy in the treatment of bile duct carcinoma.

Forty-two patients with irresectable bile duct carcinoma (n = 31) or with microscopic evidence of tumor rest after aggressive surgery for bile duct carcinoma (n = 11) were given radiotherapy consisting intentionally of external-beam therapy and intraluminal 192Iridium (192Ir) wire application(s) following bile drainage procedures. The treatment was well tolerated; complications were mainly infectious and related to the success of the drainage. A median survival of 10 months was achieved for the group as a whole. Patients treated following microscopically incomplete resection survived longer than patients with an irresectable tumor (15 vs 8 months median survival, p = 0.06). Gross lymph node involvement also proved to be a prognostic factor.

Intrahepatic cholesterol stones associated with peripheral cholangiocellular carcinoma: an autopsy case.

A rare autopsy case of intrahepatic cholesterol stones associated with peripheral intrahepatic cholangiocellular carcinoma is presented. The patient, a 60-yr-old man, was diagnosed as having peritoneal dissemination of adenocarcinoma, and died of respiratory failure 4 months later. At autopsy, intrahepatic cholangiocellular carcinoma was found at the left lateral segment of the liver. The cancer was extensively disseminated to the peritoneal cavity. In addition, the liver harbored a cholesterol stone (1.0 cm in diameter) impacted in the right peripheral bile duct, as well as tiny cholesterol stones (0.1-0.2 cm in diameter) scattered in the intrahepatic peripheral bile ducts. A few tiny cholesterol stones were also present in the cancerous bile ducts. Although the causal relationship between the intrahepatic cholesterol stones and cholangiocellular carcinoma remains speculative, clinicians should be aware of this association.

[Intra-arterial infusion chemotherapy in non-resectable pancreatic cancer using angiotensin-II and implantable drug delivery system].

Over the past 6 years, we have treated 25 cases of pancreatic cancer, 6 cases of cholangioma in pancreas-head and 3 cases of cancer in duodenal papilla (2 cases Stage I, 5 cases stage II, 2 cases stage III, 25 cases stage IV). Twelve cases (10 unresectable cases, 1 hepatic metastasis case, 1 recurrent case) were treated with intra-arterial infusion chemotherapy using implantable Drug Delivery System, combined with angiotensin-II to increase the concentration of anti-cancer agents in cancer tissue. Twenty-four cases (70%) died in less than one year, so operation is not effective except for curative resection of cholangioma and duodenal papilla cancer. But exploratory laparotomy or inoperable cases given intermittent transcatheter arterial infusion chemotherapy (5-FU + ADM + MMC + angiotensin-II), showed favorable results (decrease of tumor size and pain in 2 cases; recanalization of obstruction in choledochus of 1 case). Especially trans-femoral or left subclavian arterial catheterization proved to be effective therapy for possibly giant or recurrent inoperable pancreatic cancer and hepatic metastasis. Using the drug delivery system, the technical approach to arterial infusion therapy and angiography has been readily undertaken. Quality of life has been improved, and course observation of the patient has been possible by imaging diagnosis and multidisciplinary treatment for advanced pancreatic cancer.