Biliary cystadenocarcinomas are rare cystic hepatic neoplasms. They can be thought of as a malignant counterpart of biliary cystadenomas.

Epidemiology

There is recognized increased female predilection. Its incidence peak is around 60 years of age.

Clinical presentation

The clinical symptoms are non-specific and are not distinctive from benign cystic liver lesions unless an invasive growth of the tumor occurs or distant metastases are present.

Pathology

The vast majority of these neoplasms are intrahepatic (97%) with only a small proportion being extrahepatic (3%).

Some biliary cystadenomas may rarely develop into a cystadenocarcinoma ⁹.

Radiographic features

General imaging features

Typically seen as a single multilocular cystic tumor with septal or mural nodules ^2,5. Imaging cannot reliably differentiate cystadenoma from cystadenocarcinoma, but the presence of septal nodularity may favor the diagnosis of biliary cystadenocarcinoma versus a biliary cystadenoma ^3,7.

Discrete soft tissue masses, thick and coarse calcifications, and heterogeneous CT attenuation or MR intensity within the loculi may also be seen, although these features are non-specific.

Contrast-enhanced ultrasound demonstrates minimal enhancement since the tumors are largely avascular ¹⁰. Abnormally increased vascularity may be present peripherally or in the septa.

At MRI, subtraction images may prove helpful for discerning faint mural or septal enhancement.

Treatment and prognosis

Surgical removal of the tumor by complete excision is often the treatment of choice. The overall prognosis is considered better than for other malignant tumors of the liver ⁸.