A PATIENT WITH SIMPSON-GOLABI-BEHMEL-SYNDROME AND HEPATOCELLULAR-CARCINOMA

Simpson-Golabi-Behmel Syndrome (SGBS) is an X linked disorder characte rised by pre- and postnatal overgrowth, coarse facial features, and vi sceral and skeletal abnormalities. Like other overgrowth syndromes, in the SGBS there is an increased risk for developing neoplasia, mainly embryonic, such as Wilms tumour. We report a 3 year old male patient w ith SGBS and hepatocellular carcinoma, a previously undescribed tumour associated with the syndrome.