Growth Hormone Excess in FD/MAS: What to Do After Total Hypophysectomy Has Failed
This protocol covers the management of growth hormone excess associated with fibrous dysplasia and MAS (FD/MAS) in patients who have already undergone total hypophysectomy and have not reached adequate hormonal control.
Previous Treatment — Failure Condition
Total hypophysectomy was performed at the prior line — the whole gland is typically involved in FD/MAS, and removing the adenoma alone is not sufficient to control growth hormone overproduction. Despite this intervention, the target IGF-1 Z-score between −2 and +1 was not achieved, warranting escalation to the present protocol.
Therapeutic Goal
The clinical objective remains the same: achieving an IGF-1 Z-score between −2 and +1.
Next-Line Approach — Partial Overview
At this stage, given risks specific to fibrous dysplasia of the skull base, the approach involves a radiation-based intervention considered only as a final recourse. The complete protocol defines the precise conditions, sequencing, and monitoring requirements for this step.
References
DOI: 10.1186/s13023-019-1102-9
- Maximal medical therapy is standard of care, and pituitary radiation should be a final recourse due to the risk of malignant transformation of skull base FD.
- 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.
- The treatment goals are to achieve an IGF-1 Z-score between −2 and +1.
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