Clinicopathological Characteristics and Prognostic Factors of Intrahepatic Biliary Cystadenocarcinoma

Abstract

Background:Surgical resection is generally considered the main curative treatment for intrahepatic biliary cystadenocarcinoma (IBCA) or suspected IBCAs, but controversy exists regarding the prognosis for IBCAs. This study aimed to describe the clinicopathological characteristics of IBCA and identify prognostic factors that may influence the survival of patients treated with surgical procedures.Methods:Thirty-four patients with histologically confirmed IBCA treated between January 2000 and June 2014 were included. The clinical characteristics of patients with IBCA were compared with those of 41 patients with intrahepatic biliary cystadenoma (IBC); factors that significant difference were analyzed for prognosis analysis of IBCA using multivariate/univariate Cox proportional hazards regression models. Survival curves were constructed using the Kaplan–Meier method and compared using the log-rank test.Results:IBCAs had a strong female predominance, and the most common presenting symptoms were abdominal pain or discomfort. Compared with IBCs, IBCAs occurred in older patients, in more male patients, and were associated statistically significant abnormal increase in alanine aminotransferase (P = 0.01) and total bilirubin (P = 0.04). Mural nodules were more frequently seen with IBCAs and may associate with malignancy. It was difficult to differentiate between IBC and IBCA based on laboratory examination and imaging findings. Although complete resection is recommended, enucleation with negative margins also achieved good outcomes. Median overall patient survival was 76.2 months; survival at 1, 3, and 5 years was 88.0%, 68.7%, and 45.8%, respectively. Radical resection and noninvasive tumor type were independent prognostic factors for overall survival.Conclusions:It remains difficult to distinguish between cystadenomas and cystadenocarcinomas based on laboratory examination and image findings. Complete resection is recommended for curative treatment, and patients should be closely followed postoperatively, particularly those with invasive tumors.

Uncommon neoplasms of the biliary tract: radiological findings

“…On CT examination, the area is hypodense; while in MRI, the intracystic signal on T 1 and T 2 W is reported as variable, in relation to the concentration of protein and heme degradation products, most commonly low signal on T 1 W and high on T 2 W, as all the cystic lesions. 20 In our series, cystadenomas appeared as hypointense on T 1 W and lower hyperintense than gallbladder on T 2 W images (Figure 4). The presence of mural contrast enhancement is not specific ( Figure 5).…”

“…The presence of mural contrast enhancement is not specific ( Figure 5). The mural nodule is an element that must be readily recognized to suspect transformation in cystadenocarcinoma 20 (Figure 6). The differential diagnosis between cystadenoma and cystadenocarcinoma is not easy.…”

“…Therefore, surgical resection is recommended for all cystic lesions suspicious for cystadenoma. 20 Recently, the term of intraductal papillary neoplasm of the biliary tract (BT-IPMN) has been proposed to describe a tumour of the biliary tract, characterized by the presence of intraluminal papillary lesions, with a fibrovascular core inside of dilated bile ducts. 21 The neoplastic cells produce an excess of mucin.…”

An updated review of cystic hepatic lesions

“…Higher enzyme levels in BCAC occur due to the invasion of the adjacent biliary tree, while BCA is a slow-growing tumor and causes late biliary compression. Serum CEA and CA 19-9 are moderate predictors of BCAC [ 31 ].…”

“…Non-invasive lesions have low recurrence and a good long-term outcome. For invasive lesions, the 5-year survival was similar to cholangiocarcinoma at around 22% [ 31 ].…”