Biliary cystadenocarcinoma
Biliary cystadenocarcinomas are rare cystic hepatic neoplasms. They can be thought of as a malignant counterpart of biliary cystadenomas.
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Epidemiology
There is recognized increased female predilection. Its incidence peak is around 60 years of age.
Clinical presentation
The clinical symptoms are nonspecific 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 a small proportion 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 nonspecific.
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.
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Hepatobiliary pathology
- depositional disorders
- infection and inflammation
- liver abscess
- hepatic hydatid infection
- cirrhosis
- hepatitis
- cholecystitis
- cholangitis
- malignancy
- liver and intrahepatic bile duct tumors
- benign epithelial tumors
- hepatocellular hyperplasia
- hepatocellular adenoma
- hepatic/biliary cysts
- benign nonepithelial tumors
- primary malignant epithelial tumors
- hepatocellular carcinoma
- hepatocellular carcinoma variants
-
cholangiocarcinoma
- intra-hepatic
- mass-forming type
- periductal infiltrating type - Klatskin tumors
- intraductal growing type
- extra-hepatic/large duct type
- intra-hepatic
- biliary cystadenocarcinoma
- combined hepatocellular and cholangiocarcinoma
- hepatoblastoma
- undifferentiated carcinoma
- primary malignant nonepithelial tumors
- hematopoietic and lymphoid tumors
- primary hepatic lymphoma
- hepatic myeloid sarcoma (hepatic chloroma)
- secondary tumors
- miscellaneous
- adrenal rest tumors
- hepatic carcinosarcoma
- hepatic fibroma
- hepatic Kaposi sarcoma
- hepatic lipoma
- hepatic mesenchymal hamartoma
- hepatic myxoma
- hepatic rhabdoid tumor
- hepatic solitary fibrous tumor
- hepatic teratoma
- hepatic yolk sac tumor
- inflammatory myofibroblastic tumor (inflammatory pseudotumor)
- nodular regenerative hyperplasia
- pancreatic rest tumors
- primary hepatic carcinoid
- benign epithelial tumors
- liver and intrahepatic bile duct tumors
- metabolic
- trauma
-
vascular and perfusion disorders
- portal vein related
- hepatic artery related
- hepatic veins related
- inferior vena cava related
- other
- third inflow
- liver thrombotic angiitis
- infra diaphragmatic total anomalous pulmonary venous return (TAPVR)
- hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease)