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A patient with Simpson-Golabi-Behmel syndrome and hepatocellular carcinoma.

ABSTRACT: Simpson-Golabi-Behmel syndrome (SGBS) is an X linked disorder characterised by pre- and postnatal overgrowth, coarse facial features, and visceral 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 with SGBS and hepatocellular carcinoma, a previously undescribed tumour associated with the syndrome.

SUBMITTER: Lapunzina P

PROVIDER: S-EPMC1051222 | biostudies-other | 1998 Feb

REPOSITORIES: biostudies-other

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A patient with Simpson-Golabi-Behmel syndrome and hepatocellular carcinoma.

Lapunzina P P Badia I I Galoppo C C De Matteo E E Silberman P P Tello A A Grichener J J Hughes-Benzie R R

Journal of medical genetics 19980201 2

Simpson-Golabi-Behmel syndrome (SGBS) is an X linked disorder characterised by pre- and postnatal overgrowth, coarse facial features, and visceral 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 with SGBS and hepatocellular carcinoma, a previously undescribed tumour associated with the syndrome. ...[more]

PMID: 9507397

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