LIPA and cholangiocarcinoma: Although the cholesteryl esters and triglycerides are deposited in hepatocytes and Kupffer cells, distinct progression to malignancies has been reported: a girl with HCC (11y),[13] a man with cholangiocarcinoma (51y),[14,15] and a man with hepatocellular-cholangiocarcinoma (21y).[16] The latter had the same mutation in the gene LIPA as our patient (allele c.386A > G homozygous p.H129R).