• Dean J Arnaoutakis, Yuhree Kim, Carlo Pulitano, Victor Zaydfudim, Malcolm H Squires, David Kooby, Ryan Groeschl, Sorin Alexandrescu, Todd W Bauer, Mark Bloomston, Kevin Soares, Hugo Marques, T Clark Gamblin, Irinel Popescu, Reid Adams, David Nagorney, Eduardo Barroso, Shishir K Maithel, Michael Crawford, Charbel Sandroussi, Wallis Marsh, and Timothy M Pawlik.
• *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.
• Ann. Surg. 2015 Feb 1; 261 (2): 361-7.

ObjectiveTo characterize clinical and radiological features associated with biliary cystic tumors (BCTs) of the liver, and to define recurrence-free and overall survival.BackgroundBiliary cystadenoma (BCA) and biliary cystadenocarcinoma (BCAC) are rare tumors that arise in the liver.MethodsBetween 1984 and 2013, 248 patients who underwent surgical resection of BCA or BCAC were identified. Clinical and outcome data were analyzed.ResultsMedian 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).ConclusionsAmong 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.

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