In fibrous dysplasia of bone, growth hormone excess associated with FD/MAS is a recognised complication requiring active management. When the previous medical regimen has not achieved adequate IGF-1 normalisation, a structured next step is indicated.
Clinical scenario: Growth hormone excess associated with FD/MAS — pituitary involvement drives persistently elevated IGF-1, requiring a treatment approach specifically tailored to this presentation.
This protocol applies after pegvisomant alone, or pegvisomant in combination with octreotide or lanreotide, has failed to bring IGF-1 to a Z-score between −2 and +1. When that goal remains out of reach on this regimen, escalation to a further intervention is indicated.
The protocol at this stage involves pituitary surgery, with the extent of the surgical intervention reflecting the nature of glandular involvement in FD/MAS. The complete protocol — including candidacy criteria, clinical sequencing, and all procedural detail — is available via the structured regimen below.
Treatment goal: IGF-1 Z-score between −2 and +1.
DOI: 10.1186/s13023-019-1102-9
Somatostatin analogues are first line therapies with second line options including pegvisomant, alone or in combination with octreotide or lanreotide at the discretion of the treating physician.
Pituitary surgery is recommended for patients resistant to medical therapy.
Total hypophysectomy is required as the whole gland is usually involved and removing just the adenoma is not enough to control the excess production of growth hormone.
The treatment goals are to achieve an IGF-1 Z-score between −2 and +1.
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