Occurrence of Cranial Neoplasms in Pediatric Patients with Noonan Syndrome Receiving Growth Hormone: Is Screening with Brain MRI prior to Initiation of Growth Hormone Indicated?

Horm Res Paediatr. 2017;88(6):423-426. doi: 10.1159/000479107. Epub 2017 Jul 26.

Abstract

Noonan syndrome (NS) is associated with short stature. Growth hormone treatment has been FDA approved for use in these patients. Children with NS are at a higher risk of developing benign and malignant proliferative disorders, primary brain tumors being one of them. Since growth hormone therapy can worsen the tumor burden, screening with a brain MRI prior to growth hormone initiation in NS patients is strongly recommended. Here we present two NS patients who developed different primary brain tumors while being on growth hormone therapy.

Keywords: Brain tumors; Growth hormone; Noonan syndrome.

MeSH terms

  • Exome*
  • Female
  • Growth Disorders / genetics*
  • Humans
  • Insulin-Like Growth Factor I / deficiency*
  • Male
  • Sequence Analysis, DNA*

Substances

  • IGF1 protein, human
  • Insulin-Like Growth Factor I