Hepatocarcinoma in a Child With the Alagille Syndrome

Arteriohepatic dysplasia (Alagille's syndrome): unusual hepatic architecture and function

“…Previous reports of the various skeletal abnormalities of patients with this syndrome were summarized by Rosen field et al in 1980 and by Brunelle et al in 1986 [3, 12]. Of interest are three separate case reports of hepatocellular carcinoma in association with Alagille's syndrome [13][14][15]. In all three cases, CT scan of the liver was performed; two had MRI scans, two had sonograms, and one had a Tc99m DISIDA scan.…”

Alagille syndrome (arteriohepatic dysplasia) and del(20)(p11.2)

“…Therefore, it seems plausible to suggest that the gene(s) for Alagille syndrome is located on the short arm of chromosome 20. Moreover, the occurrence of hepatocellular carcinoma in children with Alagille syndrome [Kaufman et al, 1987;Ong et al, 1986;Rabinovitz et al, 19891 and in an adult Alagille syndrome patient without cirrhosis [Adams, 19861 has been previously explained by chronic liver disease, but in the light of the present observation one can speculate that loss of heterozygosity for a cell-cycleregulating gene may be an alternative explanation, as is observed in retinoblastoma [Hansen and Cavence, 19881.…”

Alagille syndrome and hepatocarcinoma: a case report

“…Liver cirrhosis develops in 12-14% of cases. Hepatocarcinoma is a very rare complication of this disease (1)(2)(3).…”

“…Hepatocarcinoma is a very rare event in these patients. Most cases of hepatocarcinoma in patients with Alagille syndrome follow the development of cirrhosis (2,3). The present case developed hepatocarcinoma without cirrhosis.…”