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Fulminant liver failure in a patient affected by polycystic liver disease and liver metastases from breast carcinoma

Polycystic liver disease (PLD) is a rare, congenital, benign condition characterized by the presence of multiple bile-duct-derived epithelial cysts in the liver parenchyma. The disease is usually asymptomatic, but cyst growth can result in complications such as ascites, esophageal varices, jaundice...

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Bibliographic Details
Published in:Tumori 2009-07, Vol.95 (4), p.557-561
Main Authors: Fadda, Giovanni Maria, Santeufemia, Davide Adriano, Cossu-Rocca, Paolo, Bardino, Gianfranco, Costantino, Salvatore, Sanna, Giovanni, Sarobba, Maria Giuseppa, Farris, Antonio
Format: Article
Language:English
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Summary:Polycystic liver disease (PLD) is a rare, congenital, benign condition characterized by the presence of multiple bile-duct-derived epithelial cysts in the liver parenchyma. The disease is usually asymptomatic, but cyst growth can result in complications such as ascites, esophageal varices, jaundice and hepatic failure. The exact mechanism leading to cyst growth is unclear, but estrogenic stimulation and paracrine action of vascular endothelial growth factor (VEGF) are thought to play a role in the growth of cyst epithelium. We report a case of acute liver failure in a young woman with PLD and liver metastases from breast carcinoma. No data are available in the literature about metastatic liver involvement in PLD patients affected by breast cancer. The prognosis of patients with liver metastases is generally poor but fulminant liver failure is a very rare occurrence. Estrogen stimulation seems to be a risk factor for breast cancer and severe PLD. In the reported case, the presence of either the cysts or the metastatic lesions may have resulted in more extensive liver damage. The adoption of drugs selected in relation to their hepatic toxicity together with careful monitoring of liver function is warranted in the management of breast cancer patients affected by PLD, in order to reduce the risk of liver failure.
ISSN:0300-8916
2038-2529
DOI:10.1177/030089160909500430