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Chirurgia 2006 February;19(1):79-85

language: English

Autosomal dominant polycystic liver disease. Case report and new outcomes

De Cecco C. N. 1, Mafrici M. 1, Musciano V. 1, Barbetta F. 1, Impallara D. 1, Venditti C. 1, Pacchiarotti A. 2, Alvaro D. 3, Battisti G. 4

1 Università “La Sapienza” Roma, 1a Facoltà di Medicina e Chirurgia, Polo Pontino, Rome, Italy
2 U.O.C. di Gastroenterologia, Latina, Italy
3 U.O.C. di Chirurgia Generale, Latina, Italy


ADPLD (Autosomal Dominant Polycystic Liver Disease) is a hepatic disease, relatively rare, due to not very well-known genetic alterations, which cause onset of cystic lesions limited to hepatic parenchyma. The disease is transmitted with an autosomal dominant mechanism , like autosomal dominant polycystic kidney disease, and is associated with a specific genetic alteration, localized on chromosome 19p13.2-13.1. ADPLD is more severe in females and a correlation has been noticed between disease severity and pregnancy number. These observations underline the role of estrogen in the onset and development of polycystic liver disease, due to biliary epithelium proliferation and secretion caused by continuous hormonal stimulation 5-7, and suggest a common mechanism in cyst formation between ADPLD and ADPKD (Autosomal Dominant Polycystic Kidney Disease). It shows a remarkable phenotypic variability due to an uncomplete genetic penetrability, associated with few symptoms compared to polycystic kidney disease. The presentation of a cyst case in our hospital gave us the -opportunity to start a review on the etiopathogenesis, treatments and links with similar diseases in other organs. It made us also reflect about hepatic cyst classifications, noticing, in our judgement, a certain lack of uniformity and mostly a certain tendency to a “forced” gathering of different diseases. This consideration drove us toward the formulation of a new classification, based on morphostructural characteristics, etiologies and different therapeutic approaches .

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