Acromegaly
ICD-10 E22.0 · ICD-11 5A60.0

Treatment of Acromegaly Presenting as Gigantism Arising During Childhood or Puberty

Clinical Scenario

This protocol addresses the rare presentation of gigantism caused by a GH-secreting pituitary adenoma arising during childhood or puberty — specifically before epiphyseal closure.

Because GH hypersecretion occurs while the growth plates remain open, it results in excessive linear growth alongside the biochemical features driven by both elevated GH and IGF-1 levels.

Approach

Management targets normalization of GH and IGF-1 hypersecretion. The first-line intervention is directed at the pituitary tumor itself.

The full evidence-based regimen — including sequencing, adjuvant considerations, and follow-up targets — is available in the complete protocol below.

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References

Gigantism is caused by very rarely encountered sporadic or familial GH-secreting adenomas arising during childhood or puberty.

GH hypersecretion occurring before epiphyseal closure results in excessive linear growth and phenotypic features of gigantism due to both elevated GH and IGF-1 levels.

Surgery is the first line of therapy, although adjuvant therapy is often required.

In patients with the rare presentation of gigantism, we recommend the standard approaches to normalizing GH and IGF-1 hypersecretion as described elsewhere in this guideline.

DOI: 10.1210/jc.2014-2700

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