Management of Biliary Cystic Tumors: A Multi-institutional Analysis of a Rare Liver Tumor

• Type of Material: Article
• Authors: Dean J. Arnaoutakis, Johns Hopkins University; Yuhree Kim, Johns Hopkins University; Carlo Pulitano, Royal Prince Alfred Hospital; Victor Zaydfudim, Mayo Clinic; Malcolm H. Squires, Emory University; David Kooby, Emory University; Ryan Groeschl, Medical College of Wisconsin; Sorin Alexandrescu, Fundeni Institute; Todd W. Bauer, University of Virginia; Mark Bloomston, Ohio State Wexner Medical Center; Kevin Soares, Johns Hopkins University; Hugo Marques, Curry Cabral Hospital; T. Clark Gamblin, Medical College of Wisconsin; Irinel Popescu, Fundeni Institute; Reid Adams, University of Virginia; David Nagorney, Mayo Clinic; Eduardo Barroso, Curry Cabral Hospital; Shishir Maithel, Emory University; Michael Crawford, Royal Prince Alfred Hospital; Charbel Sandroussi, Royal Prince Alfred Hospital; Wallis Marsh, University of Pittsburgh; Timothy M. Pawlik, Johns Hopkins University
• Language: English
• Date: 2015-02-01
• Publisher: Lippincott, Williams & Wilkins
• Publication Version: Author Accepted Manuscript (After Peer Review)
• Copyright Statement: Copyright © 2014 Wolters Kluwer Health, Inc. All rights reserved.
• Final Published Version (URL): http://dx.doi.org/10.1097/SLA.0000000000000543
• Title of Journal or Parent Work: Annals of Surgery
• ISSN: 0003-4932
• Volume: 261
• Issue: 2
• Start Page: 361
• End Page: 367
• Keywords: Surgery; BENIGN TUMORS; STROMA; Science & Technology; HEPATOBILIARY CYSTADENOMA; cyst; EXPERIENCE; CYSTADENOCARCINOMA; NEOPLASMS; biliary cystadenoma; Life Sciences & Biomedicine; biliary cystadenocarcinoma; liver
• Research Categories: Health Sciences, Medicine and Surgery; Health Sciences, Oncology

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.