title: Management of biliary cystic tumors: a multi-institutional analysis of a rare liver tumor.
abstract: OBJECTIVE
  To characterize clinical and radiological features associated with biliary cystic tumors (BCTs) of the liver, and to define recurrence-free and overall survival.
  
  
  BACKGROUND
  Biliary cystadenoma (BCA) and biliary cystadenocarcinoma (BCAC) are rare tumors that arise in the liver.
  
  
  METHODS
  Between 1984 and 2013, 248 patients who underwent surgical resection of BCA or BCAC were identified. Clinical and outcome data were analyzed.
  
  
  RESULTS
  Median total bilirubin, CA19-9, and carcinoembryonic antigen (CEA) levels were 0.6 mg/dL, 15.0 U/mL, and 2.7 ng/mL, respectively. Preoperative imaging included computed tomography only (62.5%), magnetic resonance imaging only (6.9%), or CT + MRI (18.5%). Features on cross-sectional imaging included multiloculation (56.9%), mural nodularity (16.5%), and biliary ductal dilatation (17.7%). The presence of these factors did not reliably predict BCAC versus BCA (sensitivity, 81%; specificity, 21%). Median biliary cyst size was 10.0 cm (interquartile range, 7-13 cm). Operative interventions included unroofing/partial excision of the lesion (14.1%), less than hemihepatectomy (48.8%), or hemi-/extended hepatectomy (36.3%). On pathology most lesions were BCA (89.1%), whereas 27 (10.9%) were BCAC. At last follow-up, there were 46 (18.3%) recurrences; 2 patients who initially had BCA recurred with BCAC. Median overall survival was 18.1 years; 1-year, 3-year, and 5-year survival was 95.0%, 86.8%, and 84.2%, respectively. Long-term outcomes were associated with BCAC versus BCA, as well as the presence of spindle cell/ovarian stroma (both P < 0.05).
  
  
  CONCLUSIONS
  Among patients undergoing surgery for BCT, associated malignancy was uncommon (10%) and no preoperative findings reliably predicted underlying BCAC. After excision of BCA, long-term outcomes were good; however, patients with BCAC had a worse long-term prognosis.
authors: *Departments of Surgery at Johns Hopkins University School of Medicine, Baltimore, MD †Royal Prince Alfred Hospital, Sydney, Australia ‡Mayo Clinic, Rochester, MN §Emory University School of Medicine, Atlanta, GA ¶University of Pittsburgh, Pittsburgh, PA ‖Medical College of Wisconsin, Milwaukee, WI **Fundeni Institute, Bucharest, Romania ††University of Virginia, Charlottesville, VA ‡‡Ohio State Wexner Medical Center, Columbus, OH; and §§Curry Cabral Hospital, Lisbon, Portugal.
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mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001650:Bile Duct Neoplasms / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D001653:Bile Ducts, Intrahepatic / Q000601:surgery*; D003536:Cystadenocarcinoma / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D003537:Cystadenoma / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D016208:Databases, Factual; D005260:Female; D005500:Follow-Up Studies; D006498:Hepatectomy; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D016019:Survival Analysis; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D055815:Young Adult