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Growth Hormone Excess in Children with Optic Pathway Tumors Is a Transient Phenomenon

Jami L Josefson1, Robert Listernick, Joel Charrow

  • 1Division of Endocrinology, Ann and Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Ill., USA.

Hormone Research in Paediatrics
|June 28, 2016
PubMed

Insights

Growth hormone (GH) excess in children with optic pathway tumors (OPT) is often overlooked. In a study of 7 children, GH excess resolved in most cases, suggesting treatment benefits may be unclear.

Area of Science:

  • Pediatric Endocrinology
  • Neuro-oncology
  • Genetics

Background:

  • Growth hormone (GH) excess is underrecognized in children with chiasmal optic pathway tumors (OPT), frequently linked to neurofibromatosis type 1 (NF1).
  • These children exhibit elevated insulin-like growth factor 1 (IGF-1) and rapid growth despite precocious puberty treatment.
  • GH levels fail to suppress after an oral glucose challenge in affected children.

Purpose of the Study:

  • To describe the treatment course and natural history of GH excess in children with OPT.
  • To investigate the clinical presentation and biochemical evidence of GH excess in this pediatric population.

Main Methods:

  • A descriptive case series included 7 children (5 previously described, 2 newly diagnosed).
  • All patients presented with clinical and biochemical signs of GH excess.
  • Treatment involved the somatostatin analog octreotide.

Main Results:

  • Treatment duration varied among the 7 patients.
  • Five out of seven patients achieved resolution of GH excess.
  • These 5 patients normalized IGF-1 levels without ongoing treatment.

Conclusions:

  • A subset of children with OPT experience unrestrained GH secretion with potential adverse outcomes.
  • GH excess appears to resolve spontaneously over time in these patients.
  • The benefit of interventions like octreotide in altering outcomes or preventing tumor progression remains uncertain.
Abstract

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