A rare case of cholangiocarcinoma and dysgerminoma synchronously associated with Turner's syndrome was reported. A 53-year-old woman was admitted to our hospital on July 12, 1984 due to intrahepatic and left inguinal tumors. Physical examination revealed the typical characteristics of Turner's syndrome. The karyotype from myelocyte and fibrocyte culture was interpreted as 45, X. The resected intrahepatic tumor was cholangiocarcinoma that had invaded to the transverse mesocolon and the duodenum, while the inguinal lesion was dysgerminoma derived from a dysgenetic gonad. As far as we have been able to ascertain through our investigations of the literature on malignant disease in Turner's syndrome, the association of cholangiocarcinoma with Turner's syndrome has not been reported previously.
Limiting intraoperative blood loss and the consequent need for whole blood transfusion is a widely accepted goal in liver resection. To achieve this goal, liver resection carried out under warm ischemia seems adequate. Methods used for this purpose can be categorized as follows: those with only an inflow control and the one that consists of total vascular exclusion. Liver resections under inflow vascular control are safer than those performed without. Furthermore, up to now the ischemia-reperfusion liver damage does not seem to affect the patients' course. The clinical evidence shows that intermittent warm ischemia seems to be safer than the continuous clamping and guarantees an effective control of the intraoperative bleeding. Conversely, total vascular exclusion is an invasive technique with not negligible morbidity; then its real indications should be restricted to exceptional cases, such as those with infiltration of the inferior vena cava which demands substitution of the involved vessel. In conclusion, up to now intermittent warm ischemia is the most appropriate approach to carry out safe liver resections.
A 70-year-old man was admitted on July 18, 1984, due to bile duct carcinoma at the porta hepatis after percutaneous drainage of the left hepatic duct. The tumor was located mainly in the right hepatic duct infiltrating to the left hepatic duct, the right main portal vein and the liver parenchyma, so that acute obstructive suppurative cholangitis was a complication. Although ultrasonically guided biliary drainage was performed and three catheters were inserted in the right intrahepatic ducts, cholangitis was not controlled. Therefore, emergent right extended hepatic lobectomy was performed on July 27. At laparotomy, the right lobe of the liver was atrophic and the right lobe was compensatorily hypertrophic. As soon as laparotomy had been accomplished, the liver abscesses and the obstructed bile ducts of the right lobe were punctured and aspirated and vigorous lavage was carried out under ultrasonic guidance. He recovered almost uneventufully, was discharged on August 25, and has enjoyed normal life for eight months. This report suggests that in patients with Klatskin tumor and acute obstructive suppurative cholangitis, emergent extended lobectomy is indicated if biliary drainage is not effective.
