title: Characteristics of sarcomatous cholangiocarcinoma of the liver.
abstract: OBJECTIVE
  The aim of this study was to clarify the clinicopathological characteristics of intrahepatic cholangiocarcinoma with sarcomatous changes.
  
  
  METHODS
  Four cases of cholangiocarcinoma with sarcomatous change were identified and investigated. The clinicopathological findings, including the results of immunohistochemical staining, were investigated in comparison with those of ordinary cholangiocarcinoma.
  
  
  RESULTS
  Two of them exhibited pyrexia as the initial symptom. The serum alkaline phosphatase level in sarcomatous cholangiocarcinoma was significantly lower than that in ordinary cholangiocarcinoma. Both the serum carcinoembryonic antigen and carbohydrate 19-9 level in sarcomatous cholangiocarcinoma also tended to be lower than those in ordinary cholangiocarcinoma. The carcinomatous component of all tumors was mostly poorly differentiated adenocarcinoma. The associated microscopic findings were as follows: lymphocyte infiltration in the tumor, accompanied by both necrosis and extensive lymph node metastases. Three of them predominantly exhibited spindle-shaped sarcomatous changes, whereas the other case predominantly demonstrated pleomorphic-type sarcomatous changes. The sarcomatous area was positive for both vimentin, a mesenchymal marker, and for epithelial markers, furthermore, in 3 of 4 cases, the carcinoma portions were also positive for vimentin. The overall survival curves were not significantly different between the 2 groups, however, no long-term survivor was found in sarcomatous cholangiocarcinoma.
  
  
  CONCLUSIONS
  The main characteristics of cholangiocarcinoma with sarcomatous changes are considered to be as follows: 1) often demonstrating pyrexia as a symptom; 2) not always demonstrating remarkable abnormal findings in the laboratory data including tumor markers; 3) histologically showing poorly differentiated adenocarcinoma; and 4) showing a very poor prognosis (especially, in a pleomorphic-type).
authors.affiliation: Department of Surgery II, Faculty of Medicine, Kyushu University, Fukuoka, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Shimada
authors.forename: K
authors.initials: K
authors.lastname: Takenaka
authors.forename: T
authors.initials: T
authors.lastname: Rikimaru
authors.forename: T
authors.initials: T
authors.lastname: Hamatsu
authors.forename: Y
authors.initials: Y
authors.lastname: Yamashita
authors.forename: K
authors.initials: K
authors.lastname: Kajiyama
authors.forename: K
authors.initials: K
authors.lastname: Taguchi
authors.forename: K
authors.initials: K
authors.lastname: Shirabe
authors.forename: K
authors.initials: K
authors.lastname: Sugimachi
mesh_terms: D000368:Aged; D001650:Bile Duct Neoplasms / Q000473:pathology* / Q000601:surgery; D001653:Bile Ducts, Intrahepatic / Q000473:pathology* / Q000601:surgery; D016009:Chi-Square Distribution; D018281:Cholangiocarcinoma / Q000473:pathology* / Q000601:surgery; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008111:Liver Function Tests; D008297:Male; D008875:Middle Aged; D012509:Sarcoma / Q000473:pathology* / Q000601:surgery; D018709:Statistics, Nonparametric; D016896:Treatment Outcome