Cystic Disease of the Liver

Abstract

Liver cysts are common. Nearly all patients are asymptomatic as these cysts are identified incidentally during ultrasonographic or cross-sectional imaging for unrelated symptoms. The vast majority of liver cysts are simple cysts, with an estimated prevalence ranging from 5% to 20% in the general population. They occur in the absence of known genetic mutations or hereditary predisposition; polycystic liver diseases, which have a hereditary component, are considerably rarer. Polycystic liver disease has a phenotypic spectrum from numerous to innumerable simple cysts, is an autosomal dominant trait and most commonly occurs in patients with autosomal dominant polycystic kidney disease. Asymptomatic, simple, or polycystic liver cysts do not require intervention, while management of symptomatic cysts depends on patient presentation, extent of cystic disease, and available center-specific treatment options. Patients with polycystic liver disease should be evaluated and treated at experienced referral centers using a multidisciplinary approach. Neoplastic liver disease, including cystadenoma, cystadenocarcinoma, and intraductal papillary mucinous neoplasms of the bile duct (IPMN-B), also is rare. The carcinogenesis pathway between cystadenoma and cystadenocarcinoma is poorly understood. The decision to proceed with resection or surveillance of cystadenoma is multifactorial and depends on patient and imaging specific factors. IPMN-B has a high risk of malignant transformation or malignancy on presentation and resection is recommended. Other etiologies of cystic disease are rare and are frequently cystic primary or secondary malignancies (such as cystic-appearing hepatocellular carcinoma, cystic-appearing primary liver sarcoma, cystic colorectal, neuroendocrine or other metastases), with features masquerading as simple cysts. Appropriate treatment depends on the following: (1) making definitive and correct diagnosis; (2) if symptomatic, determining whether symptoms are related to the cyst(s); and (3) selecting the best individualized treatment option for each patient.