When Adjuvant Medical Therapy Has Not Controlled GH and IGF-1 Hypersecretion in Gigantism Arising During Childhood or Puberty

Clinical Scenario

Gigantism in this context is caused by a GH-secreting pituitary adenoma that arose during childhood or puberty — before epiphyseal closure. GH hypersecretion prior to epiphyseal closure drives excessive linear growth through both elevated GH and IGF-1 levels.

Previous Treatment — Insufficient Response

Adjuvant medical therapy with octreotide LAR, or pegvisomant in those resistant to somatostatin receptor ligands, did not achieve normalization of GH and IGF-1 hypersecretion or adequate control of growth velocity. This protocol addresses the recommended next step.

Treatment Goals

Normalization of GH and IGF-1 hypersecretion.

Approach — Partial Overview

The structured regimen includes radiation therapy as an adjuvant modality directed at the pituitary tumor mass and hormone hypersecretion. Management in this population may require a combination of interventions. The complete evidence-based regimen is available via the link below.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1210/jc.2014-2700

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.

Accordingly, management approaches including treatment combinations of more than one surgical procedure, combined medical treatments, and RT may all be 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.

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