Hepatobiliary Cystadenomas and Cystadenocarcinomas

A Report of 33 Cases

Xinting Sang; Yongliang Sun; Yilei Mao; Zhiying Yang; Xin Lu; Huayu Yang; Haifeng Xu; Shouxian Zhong; Jiefu Huang

Disclosures

Liver International. 2011;31(9):1337-1344. 

In This Article

Abstract and Introduction

Abstract

Background: Hepatobiliary cystadenomas and cystadenocarcinomas are rare and often misdiagnosed.
Aims: We report our experience with 33 cases over 20 years to discuss an algorithm for these diseases.
Methods: Patients presenting with a diagnosis of hepatobiliary cystadenomas and cystadenocarcinomas were retrospectively reviewed from January 1991 to October 2010. Clinical data were collected by examining hospital records and by follow-up questionnaire interviews.
Results: Thirty-three patients had pathologically diagnosed hepatobiliary cystadenomas (19/33, 17 females and two males) or cystadenocarcinomas (14/33, five females and nine males). Symptoms of cystadenomas at hospitalization were abdominal bloating or pain (9/19). Nine patients had an elevated level of carbohydrate antigen (CA) 19-9. The surgical procedures, i.e. cyst enucleation, segmentectomy, sectionectomy and hemihepatectomy, were performed with satisfactory outcomes. Symptoms of cystadenocarcinomas included abdominal bloating or pain (8/14) and fever (3/14). Seven patients had elevated CA19-9. The imaging characteristics of cystadenocarcinomas were similar to those of cystadenomas. The clinical outcomes for cystadenocarcinomas were mostly poor after either surgical or conservative treatment.
Conclusions: Clinical symptoms are unreliable for these diagnoses and their differential diagnosis. Imaging evaluations and CA19-9 are of value for the recognition of cystadenoma and cystadenocarcinoma, but not for their differential diagnosis. Any recurrence of liver cyst after surgery or other treatments should lead one to suspect one of these diseases. Invasive examination and percutaneous fine-needle aspiration cytology are not recommended. Complete excision or careful enucleation should be the first treatment choice for a better prognosis.

Introduction

Hepatobiliary cystadenomas and cystadenocarcinomas are rare neoplasms, with the first case having been reported in 1892 by Keen.[1] They account for 1% of liver cystic lesions and approximately 5% of symptomatic hepatic cysts.[2–4] Because of their rarity and presentations similar to those of other cystic liver lesions, the diagnosis is often delayed; this results in inappropriate treatment modalities, such as aspiration, causing unnecessary morbidity and mortality.[5,6] A second-stage operation is sometimes needed because of the misdiagnosis of the disease. Reports of these diseases have been increasing because of advances in imaging techniques such as computed tomography (CT) and magnetic resonance imaging (MRI) in recent years. Here, we present our experience with 19 hepatobiliary cystadenomas and 14 cystadenocarcinomas over the last 20 years in a leading teaching hospital in China, offer a pertinent review of the literature and discuss current treatment modalities. This series is one of the largest clinical reports in the published literature.

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