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A patient with Simpson-Golabi-Behmel syndrome and hepatocellular carcinoma.
  1. P Lapunzina,
  2. I Badia,
  3. C Galoppo,
  4. E De Matteo,
  5. P Silberman,
  6. A Tello,
  7. J Grichener,
  8. R Hughes-Benzie
  1. Department of Paediatrics, Hospital de Niños de Buenos Aires, University of Buenos Aires, Argentina.

    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.

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