title: Clinical studies of mucin-producing cholangiocellular carcinoma: a study of 22 histopathology-proven cases.
abstract: OBJECTIVE
  We present the clinical features and outcomes of 22 surgically treated and histopathology-proven cases of mucin-producing cholangiocellular carcinoma (MPCCC).
  
  
  BACKGROUND
  Cholangiocellular carcinoma (CCC) is an uncommon malignancy. Unlike hepatocellular carcinoma, it is difficult to set up a high-risk group, and a specific tumor marker has yet to be found. Chronic liver disease is usually not found to be associated with CCC. Information about patients with MPCCC is limited, and the frequency of MPCCC in all patients with CCC has not been reported.
  
  
  METHODS
  The clinical features of 22 surgically treated and histopathology-proven cases of MPCCC were reviewed, including morbidity, mortality, and follow-up results. Factors that may influence the outcomes were also analyzed. Clinical features and outcomes of 148 patients with non-mucin-producing cholangiocellular carcinoma (non-MPCCC) were also summarized for comparison.
  
  
  RESULTS
  Of 170 cases of CCC, 22 (12.9%) were MPCCC. Imaging studies were important in the differential diagnosis of CCC. Operative findings (e.g., gross appearance of the liver, mucobilia found by common bile duct exploration, choledochoscopic findings, and frozen section) were useful in the diagnosis of MPCCC. Surgical procedures included common bile duct exploration, or hepaticostomy, and intraoperative choledochoscopy in all 22 patients. Hepatic resection was done in 14 of the 22 cases (63.6%). No early surgical mortality was noted. Wound infections (two patients), bile leak (one patient), and intraabdominal abscess (one patient) were the postoperative complications. The 1-, 2-, 3-, 4-, and 5-year survival rates were 86.5%, 68.5%, 59.0%, 38.5%, and 31.0%, respectively. A significant difference in survival pattern was found between the MPCCC and non-MPCCC patient groups. Patients with hepatic resection had a significantly better prognosis than those without resection. Although patients with hepatolithiasis had a better survival pattern than those without hepatolithiasis, the difference was not statistically significant.
  
  
  CONCLUSIONS
  We present the clinical features and outcomes of 22 surgically treated and histopathology-proven cases of MPCCC. Patients with hepatic resection were found to have better survival rates.
authors.affiliation: Department of Surgery and Pathology, Chang Gung Memorial Hospital, Chang Gung Medical College, Taipei, Taiwan.
authors.forename: M F
authors.initials: MF
authors.lastname: Chen
authors.forename: Y Y
authors.initials: YY
authors.lastname: Jan
authors.forename: T C
authors.initials: TC
authors.lastname: Chen
mesh_terms: D002288:Adenocarcinoma, Mucinous / Q000473:pathology* / Q000601:surgery; D000368:Aged; D000369:Aged, 80 and over; D001650:Bile Duct Neoplasms / Q000473:pathology* / Q000601:surgery; D001653:Bile Ducts, Intrahepatic; D018281:Cholangiocarcinoma / Q000209:etiology / Q000473:pathology* / Q000601:surgery; D002760:Cholangiopancreatography, Endoscopic Retrograde; D003937:Diagnosis, Differential; D005260:Female; D005500:Follow-Up Studies; D006498:Hepatectomy / Q000009:adverse effects / Q000379:methods; D006801:Humans; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012307:Risk Factors; D016019:Survival Analysis; D016896:Treatment Outcome