Diagnosis and management of cystic lesions of the liver
- Arie Regev, MD
Arie Regev, MD
- Adjunct Associate Professor of Medicine
- Indiana University School of Medicine
- K Rajender Reddy, MD
K Rajender Reddy, MD
- Ruimy Family President's Distinguished Professor of Medicine
- Professor of Medicine in Surgery
- Director of Hepatology
- Director, Viral Hepatitis Center
- Medical Director of Liver Transplantation
- University of Pennsylvania School of Medicine
- Section Editor
- Sanjiv Chopra, MD, MACP
Sanjiv Chopra, MD, MACP
- Editor-in-Chief — Gastroenterology/Hepatology
- Section Editor — General Hepatology
- Section Editor — Gallbladder and Biliary Tract Disease
- Professor of Medicine
- Harvard Medical School
- Senior Consultant in Hepatology
- James Tullis Firm Chief
- Beth Israel Deaconess Medical Center
Cystic lesions of the liver represent a heterogeneous group of disorders, which differ in etiology, prevalence, and clinical manifestations (table 1). Most liver cysts are found incidentally on imaging studies and tend to have a benign course. A minority can cause symptoms and rarely may be associated with serious morbidity and mortality [1-3]. Larger cysts are more likely to be symptomatic and cause complications such as spontaneous hemorrhage , rupture into the peritoneal cavity  or bile duct , infection , and compression of the biliary tree [2,7]. Specific types of cysts may have unique complications such as malignant transformation in the case of a mucinous cystic neoplasm (cystadenoma), or anaphylactic shock due to a hydatid cyst. Some of these complications may occasionally mandate surgical intervention.
In some cases, predominantly cystic liver lesions may have solid areas, particularly in the setting of malignancy. Conversely, predominantly solid liver lesions may have cystic components, as may be seen with hemangiomas or tumors that have areas that are necrotic.
Considerable controversy still exists regarding the definition and classification of cystic lesions of the liver (table 1). Furthermore, consensus has not been achieved on the optimal treatment of patients with symptomatic cysts, although a number of therapeutic approaches have been described [8,9]. This topic review will provide an overview of the diagnosis and management of cystic lesions in the liver. Detailed discussions on some of the individual causes of cysts are provided on the corresponding topic reviews.
Simple cysts of the liver are cystic formations containing clear fluid that do not communicate with the intrahepatic biliary tree. Although simple cysts are found in approximately 1 percent of autopsied adults, very few become large, and even fewer cause symptoms. Their size ranges from a few millimeters to massive lesions occupying large volumes of the upper abdomen; the largest reported cyst contained 17 liters of fluid .
Simple cysts tend to occur more commonly in the right lobe and are more prevalent in women. The female-to-male ratio is approximately 1.5:1 among those with asymptomatic simple cysts, while it is 9:1 in those with symptomatic or complicated simple cysts . Huge cysts are found almost exclusively in women over 50.
- Benhamou JP, Menu Y. Non-parasitic cystic diseases of the liver and intrahepatic biliary tree. In: Surgery of the liver and biliary tract, 2nd ed, Blumgart LH (Ed), Churchill Livingstone Inc, New York 1994. p.1197.
- Gadzijev E, Dragan S, Verica FM, Jana G. Hepatobiliary cystadenoma protruding into the common bile duct, mimicking complicated hydatid cyst of the liver. Report of a case. Hepatogastroenterology 1995; 42:1008.
- Salemis NS, Georgoulis E, Gourgiotis S, Tsohataridis E. Spontaneous rupture of a giant non parasitic hepatic cyst presenting as an acute surgical abdomen. Ann Hepatol 2007; 6:190.
- Hanazaki K, Wakabayashi M, Mori H, et al. Hemorrhage into a simple liver cyst: diagnostic implications of a recent case. J Gastroenterol 1997; 32:848.
- Akriviadis EA, Steindel H, Ralls P, Redeker AG. Spontaneous rupture of nonparasitic cyst of the liver. Gastroenterology 1989; 97:213.
- Bourgeois N, Kinnaert P, Vereerstraeten P, et al. Infection of hepatic cysts following kidney transplantation in polycystic disease. World J Surg 1983; 7:629.
- Miyamoto M, Oka M, Izumiya T, et al. Nonparasitic solitary giant hepatic cyst causing obstructive jaundice was successfully treated with monoethanolamine oleate. Intern Med 2006; 45:621.
- Jones RS. Surgical management of non-parasitic liver cysts. In: Surgery of the liver and biliary tract, 2nd ed, Blumgart LH (Ed), Churchill Livingstone, London 1994. p.1211.
- Taylor BR, Langer B. Current surgical management of hepatic cyst disease. Adv Surg 1997; 31:127.
- BURCH JC, JONES HE. Large nonparasitic cyst of the liver simulating an ovarian cyst. Am J Obstet Gynecol 1952; 63:441.
- DOCKERTY MB, GRAY HK, HENSON SW Jr. Benign tumors of the liver. III. Solitary cysts. Surg Gynecol Obstet 1956; 103:607.
- Nisenbaum HL, Rowling SE. Ultrasound of focal hepatic lesions. Semin Roentgenol 1995; 30:324.
- Spiegel RM, King DL, Green WM. Ultrasonography of primary cysts of the liver. AJR Am J Roentgenol 1978; 131:235.
- Hagiwara A, Inoue Y, Shutoh T, et al. Haemorrhagic hepatic cyst: a differential diagnosis of cystic tumour. Br J Radiol 2001; 74:270.
- Regev A, Reddy KR, Berho M, et al. Large cystic lesions of the liver in adults: a 15-year experience in a tertiary center. J Am Coll Surg 2001; 193:36.
- Lin CC, Lin SC, Ko WC, et al. Adenocarcinoma and infection in a solitary hepatic cyst: a case report. World J Gastroenterol 2005; 11:1881.
- Gigot JF, Legrand M, Hubens G, et al. Laparoscopic treatment of nonparasitic liver cysts: adequate selection of patients and surgical technique. World J Surg 1996; 20:556.
- Andersson R, Jeppsson B, Lunderquist A, Bengmark S. Alcohol sclerotherapy of non-parasitic cysts of the liver. Br J Surg 1989; 76:254.
- Kairaluoma MI, Leinonen A, Ståhlberg M, et al. Percutaneous aspiration and alcohol sclerotherapy for symptomatic hepatic cysts. An alternative to surgical intervention. Ann Surg 1989; 210:208.
- Tanaka S, Watanabe M, Akagi S, et al. Laparoscopic fenestration in combination with ethanol sclerotherapy prevents a recurrence of symptomatic giant liver cyst. Surg Laparosc Endosc 1998; 8:453.
- Blonski WC, Campbell MS, Faust T, Metz DC. Successful aspiration and ethanol sclerosis of a large, symptomatic, simple liver cyst: case presentation and review of the literature. World J Gastroenterol 2006; 12:2949.
- Wittig JH, Burns R, Longmire WP Jr. Jaundice associated with polycystic liver disease. Am J Surg 1978; 136:383.
- Henne-Bruns D, Klomp HJ, Kremer B. Non-parasitic liver cysts and polycystic liver disease: results of surgical treatment. Hepatogastroenterology 1993; 40:1.
- Litwin DE, Taylor BR, Langer B, Greig P. Nonparasitic cysts of the liver. The case for conservative surgical management. Ann Surg 1987; 205:45.
- Zacherl J, Scheuba C, Imhof M, et al. Long-term results after laparoscopic unroofing of solitary symptomatic congenital liver cysts. Surg Endosc 2000; 14:59.
- Garcea G, Pattenden CJ, Stephenson J, et al. Nine-year single-center experience with nonparastic liver cysts: diagnosis and management. Dig Dis Sci 2007; 52:185.
- Fernandez M, Cacioppo JC, Davis RP, Nora PF. Management of solitary nonparasitic liver cyst. Am Surg 1984; 50:205.
- Koperna T, Vogl S, Satzinger U, Schulz F. Nonparasitic cysts of the liver: results and options of surgical treatment. World J Surg 1997; 21:850.
- Montorsi M, Torzilli G, Fumagalli U, et al. Percutaneous alcohol sclerotherapy of simple hepatic cysts. Results from a multicentre survey in Italy. HPB Surg 1994; 8:89.
- Tikkakoski T, Mäkelä JT, Leinonen S, et al. Treatment of symptomatic congenital hepatic cysts with single-session percutaneous drainage and ethanol sclerosis: technique and outcome. J Vasc Interv Radiol 1996; 7:235.
- Larssen TB, Viste A, Jensen DK, et al. Single-session alcohol sclerotherapy in benign symptomatic hepatic cysts. Acta Radiol 1997; 38:993.
- Okano A, Hajiro K, Takakuwa H, Nishio A. Alcohol sclerotherapy of hepatic cysts: its effect in relation to ethanol concentration. Hepatol Res 2000; 17:179.
- Wernet A, Sibert A, Paugam-Burtz C, et al. Ethanol-induced coma after therapeutic ethanol injection of a hepatic cyst. Anesthesiology 2008; 108:328.
- Sanchez H, Gagner M, Rossi RL, et al. Surgical management of nonparasitic cystic liver disease. Am J Surg 1991; 161:113.
- Mazza OM, Fernandez DL, Pekolj J, et al. Management of nonparasitic hepatic cysts. J Am Coll Surg 2009; 209:733.
- Diez J, Decoud J, Gutierrez L, et al. Laparoscopic treatment of symptomatic cysts of the liver. Br J Surg 1998; 85:25.
- Katkhouda N, Hurwitz M, Gugenheim J, et al. Laparoscopic management of benign solid and cystic lesions of the liver. Ann Surg 1999; 229:460.
- Martin IJ, McKinley AJ, Currie EJ, et al. Tailoring the management of nonparasitic liver cysts. Ann Surg 1998; 228:167.
- Ooi LL, Cheong LH, Mack PO. Laparoscopic marsupialization of liver cysts. Aust N Z J Surg 1994; 64:262.
- Watson DI, Jamieson GG. Laparoscopic fenestration of giant posterolateral liver cyst. J Laparoendosc Surg 1995; 5:255.
- Gamblin TC, Holloway SE, Heckman JT, Geller DA. Laparoscopic resection of benign hepatic cysts: a new standard. J Am Coll Surg 2008; 207:731.
- Loehe F, Globke B, Marnoto R, et al. Long-term results after surgical treatment of nonparasitic hepatic cysts. Am J Surg 2010; 200:23.
- Ishak KG, Willis GW, Cummins SD, Bullock AA. Biliary cystadenoma and cystadenocarcinoma: report of 14 cases and review of the literature. Cancer 1977; 39:322.
- Wheeler DA, Edmondson HA. Cystadenoma with mesenchymal stroma (CMS) in the liver and bile ducts. A clinicopathologic study of 17 cases, 4 with malignant change. Cancer 1985; 56:1434.
- Devaney K, Goodman ZD, Ishak KG. Hepatobiliary cystadenoma and cystadenocarcinoma. A light microscopic and immunohistochemical study of 70 patients. Am J Surg Pathol 1994; 18:1078.
- Di Bisceglie AM. Malignant neoplasms of the liver. In: Chiff's Diseases of the Liver, 8th ed, Schiff ER, Sorrell MF, Maddrey WC (Eds), Lippincott-Raven, Philadephia 1999. p.1281.
- Hai S, Hirohashi K, Uenishi T, et al. Surgical management of cystic hepatic neoplasms. J Gastroenterol 2003; 38:759.
- Tomimatsu M, Okuda H, Saito A, et al. A case of biliary cystadenocarcinoma with morphologic and histochemical features of hepatocytes. Cancer 1989; 64:1323.
- Iemoto Y, Kondo Y, Fukamachi S. Biliary cystadenocarcinoma with peritoneal carcinomatosis. Cancer 1981; 48:1664.
- Devine P, Ucci AA. Biliary cystadenocarcinoma arising in a congenital cyst. Hum Pathol 1985; 16:92.
- Vick DJ, Goodman ZD, Deavers MT, et al. Ciliated hepatic foregut cyst: a study of six cases and review of the literature. Am J Surg Pathol 1999; 23:671.
- Nieweg O, Slooff MJ, Grond J. A case of primary squamous cell carcinoma of the liver arising in a solitary cyst. HPB Surg 1992; 5:203.
- Pliskin A, Cualing H, Stenger RJ. Primary squamous cell carcinoma originating in congenital cysts of the liver. Report of a case and review of the literature. Arch Pathol Lab Med 1992; 116:105.
- Hsieh CB, Chen CJ, Yu JC, et al. Primary squamous cell carcinoma of the liver arising from a complex liver cyst: report of a case. Surg Today 2005; 35:328.
- Drenth JP, te Morsche RH, Smink R, et al. Germline mutations in PRKCSH are associated with autosomal dominant polycystic liver disease. Nat Genet 2003; 33:345.
- Davila S, Furu L, Gharavi AG, et al. Mutations in SEC63 cause autosomal dominant polycystic liver disease. Nat Genet 2004; 36:575.
- SIMPLE CYST
- - Imaging studies
- - Differential diagnosis
- - Histology and needle aspiration
- NONINVASIVE MUCINOUS CYSTIC NEOPLASM (CYSTADENOMA)
- Clinical manifestations
- - Imaging studies
- - Histopathology
- MUCINOUS CYSTIC NEOPLASM WITH ASSOCIATED INVASIVE CARCINOMA (CYSTADENOCARCINOMA)
- ECHINOCOCCAL CYST
- OTHER CYSTIC LESIONS OF THE LIVER
- Ciliated hepatic foregut cyst
- Primary squamous cell carcinoma
- Liver metastases
- Polycystic liver disease
- Biliary cysts
- SUMMARY AND RECOMMENDATIONS