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Cystic Neoplasms of the Liver: Biliary Cystadenoma and Cystadenocarcinoma

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Cystic Neoplasms of the Liver: Biliary Cystadenoma and Cystadenocarcinoma COLLECTIVE REVIEWS Cystic Neoplasms of the Liver: Biliary Cystadenoma and Cystadenocarcinoma Kevin C Soares, MD, Dean J Arnaoutakis, MD, Ihab Kamel, MD, PhD, Robert Anders, MD, PhD, Reid B Adams, MD, FACS, Todd W Bauer, MD, FACS, Timothy M Pawlik, MD, MPH, PhD, FACS Cystic diseases of the liver occur in about 5% to 10% presents a decade later.7-11 Although 10% of reported of the population.1 Although simple cysts are most BCTs originate in the extrahepatic biliary tree, the over- common, biliary cystic tumors (BCTs), specifically biliary whelming majority arise from the intrahepatic biliary cystadenoma (BCA) and cystadenocarcinoma (BCAC), system.12-15 Biliary cystic tumors are typically slow-growing can arise in a subset of patients. Hueter first reported lesions with a reported size that can range in diameter BCA in 1887 and Keen reported the first BCA resection from 1.5 to 35 cm.6,10,16,17 5 years later.2,3 Since that time, there has been a relative Based on the mesenchymal stroma and the epithelium paucity of data published on the surgical management resembling endodermal (primitive hepatobiliary) cells of BCTs. Because of the rarity of BCTs, many clinicians that can be seen in BCTs, Wheeler and Edmondson are unfamiliar with the diagnostic features, therapeutic hypothesized that BCAs arise from ectopic rests of embry- management, and natural history of these liver onic bile ducts.18 However, 50% of BCTs contain endo- neoplasms. We provide an evidence-based review of crine cells, suggesting that they might originate from BCTs with particular emphasis on early recognition and intrahepatic peribiliary glands.10,20 Biliary cystadenocarci- approach to management. A search of available electronic noma is thought to originate either de novo from formed databases, including MEDLINE/Pubmed, using the biliary ducts induced by ischemia and carcinogens, or from terms biliary cystadenoma, biliary cystadenocarcinoma, malignant transformation of a pre-existing BCA.18,19,21 and non parasitic hepatic cysts was conducted. Addition- The latter theory is supported by the multiple reports of ally, we searched the MeSH database under the heading benign epithelium within BCAC specimens.8,10,18,21,22 For “Liver Neoplasm” in combination with the terms example, Devaney and colleagues10 reported malignant mentioned and Boolean operator AND or OR. Criteria transformation in 6 of 18 BCACs in their series, as well for inclusion included English-language articles using as cytological atypia/dysplasia in a number of BCAs. human subjects (Fig. 1). PRESENTATION AND DIFFERENTIAL INCIDENCE AND EPIDEMIOLOGY DIAGNOSIS CONSIDERATIONS Biliary cystic tumors, such as BCA and BCAC, constitute The clinical presentation of BCTs can vary considerably. < 4 5% of all liver cysts. Although BCA occurs predomi- Many patients with BCTs will be asymptomatic; other nantly in females (90%), BCAC is more evenly distrib- patients with BCTs might present with nonspecific symp- 2,5-10 uted between males and females. Mean age at toms, most commonly abdominal pain and distention presentation of BCA is 45 years, and BCAC typically (55% to 90%).3,7,11,17,18,23 Although laboratory values are normal in most patients, approximately 20% of patients CME questions for this article available at present with elevated liver function tests, such as an 19,24 http://jacscme.facs.org abnormal bilirubin level. Obstructive jaundice and chol- angitis are rare and do not correlate with malignant disease, Disclosure Information: Authors have nothing to disclose. Timothy J Eberlein, 12,25-30 Editor-in-Chief, has nothing to disclose. and typically occur with extrahepatic BCT. Hemor- rhage and cyst rupture are other recognized, yet very Received July 9, 2013; Revised August 18, 2013; Accepted August 20, 31-33 2013. uncommon, presenting complications of these lesions. From the Division of Surgical Oncology, Department of Surgery (Soares, The differential diagnosis of patients with complex Arnaoutakis, Pawlik), Departments of Radiology (Kamel) and Pathology cystic lesions of the liver includes BCT, as well as hydatid (Anders), The Johns Hopkins University School of Medicine, Baltimore, MD, and Division of Surgical Oncology, Department of Surgery, Univer- cyst, post-traumatic cyst, liver abscess, polycystic disease, sity of Virginia Health System, Charlottesville, VA (Adams, Bauer). hemorrhagic cyst, embryonal sarcoma, primary or meta- Correspondence address: Timothy M Pawlik, MD, MPH, PhD, FACS, static necrotic neoplasm, atypical simple cyst, and biliary Division of Surgical Oncology, Department of Surgery, The Johns Hopkins 11,24 University School of Medicine, Blalock 688, 600 N Wolfe St, Baltimore, intraductal papillary mucinous neoplasm (IPMN). MD 21287. email: [email protected] Biliary IPMN is a recently recognized entity characterized ª 2014 by the American College of Surgeons ISSN 1072-7515/13/$36.00 Published by Elsevier Inc. 119 http://dx.doi.org/10.1016/j.jamcollsurg.2013.08.014 120 Soares et al Biliary Cystadenoma and Cystadenocarcinoma J Am Coll Surg mucin is more characteristic of biliary IPMN.24,36,38 Abbreviations and Acronyms Despite management principles similar to BCT, preoper- BCA ¼ biliary cystadenoma ative identification of cystic biliary IPMN can alert the BCAC ¼ biliary cystadenocarcinoma surgeon to its typical superficial spreading tumor growth ¼ BCT biliary cystic tumor pattern.34,38 CA 19-9 ¼ carbohydrate antigen 19-9 IPMN ¼ intraductal papillary mucinous neoplasm IMAGING by mucin production and prominent intraductal papillary Cross-sectional imaging with either CT or MRI can accu- proliferation.34,35 Biliary IPMN occurs equally in both rately define and characterize simple cystic lesions. In sexes, with a mean age of 58 years.34,36 Some authors contrast, the accuracy of cross-sectional imaging to diag- now advocate classifying BCTs with biliary tree commu- nose the different types of complex cystic lesions can nication and no ovarian stroma as biliary IPMNs.23,35-37 be relatively low. Certain radiographic findings can be In one study, Zen and colleagues35 identified 9 cases of helpful when trying to differentiate BCT from other BCT, noting 5 tumors with direct communication to non-neoplastic pathologies. Specifically, ultrasound, CT, the biliary tree. None of these 5 cases contained and MRI combined with clinicopathological features ovarian-like stroma and all had clinicopathologic charac- can aid in preoperative differentiation and characteriza- teristics different from BCT.35 In a separate study, Naka- tion of hepatic cystic tumors. gawa and colleagues24 were able to preoperatively Sonographically, BCTs are anechoic with thickened differentiate BCA from biliary IPMN based on 3 main irregular walls and internal septations (Table 1).8,16,39 imaging characteristics. First, BCAs typically have Septal thickening, papillary infolding, and mural nodules a smooth tumor wall with septa inside the tumor, result- are characteristic of BCTs.40,41 Biliary cystadenocarci- ing in a “cysts-in-cyst” appearance. Second, papillary noma is more likely to contain mural or septal nodules projections are typical in IPMN and less so in BCA. and papillary projections.42-44 In the series by Seo and Third, demonstration of cystic tumor and intrahepatic colleagues,45 the authors described their experience with bile duct communication along with the presence of distal 20 BCT and 19 resected simple cysts that mimicked 1,448 records identified 23 records identified from through PubMed and MESH manual search of references of database search articles 661 records after duplicates removed 661 records screened 532 records excluded 129 Full-text articles Full-text articles assessed for eligibility excluded, with reasons (n=41) 41 non-English articles 88 studies included in qualitative synthesis Figure 1. A PRISMA diagram defining the method of inclusion and exclusion for studies used in the review. Vol. 218, No. 1, January 2014 Soares et al Biliary Cystadenoma and Cystadenocarcinoma 121 Table 1. Radiologic Characteristics of Cystic Liver Lesions Biliary cystic tumors Hepatic simple cysts Echinococcal cysts Multiloculated cyst Anechoic Daughter cyst within main cyst Internal septation Smooth borders Intracystic debris Enhancing cyst wall No perceptible wall Low signal intensity rim on T2-weighted MRI Calcifications No septations Papillary wall nodules No enhancement on CT with IV contrast Thickened irregular wall Water attenuation on CT Enhancement on CT with IV contrast Water attenuation on CT BCT. Biliary cystadenocarcinoma was associated with resonance imaging characterizes cyst fluid content by intracystic debris, bile duct dilation, and mural nodules.45 varying signal intensities on T1-weighted images, Computed tomography and ultrasound are complemen- depending on cyst fluid protein content.52 Linear low- tary modalities in evaluating BCTs.46 Sonography is signal intensity within high-intensity cysts identifies sep- more sensitive for detecting septa in cystic lesions, and tations on T2-weighted images.16 Magnetic resonance CT more accurately demonstrates size and anatomic imaging demonstrates the anatomic relationships within extent of these lesions.33,43 On CT, BCT lesions are iso- the liver and can aid surgical planning.53 The addition dense to water (<30 HU) with nodular areas enhanced of diffusion-weighted MR to conventional MRI with IV contrast.8,16,30,39,47 Biliary duct dilation, single sequences aids in the qualitative and quantitative assess- cysts, and lesions in the left lobe of the liver can be predic- ment
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