Figure 15.

Figure 15.

Recommended management for growth hormone excess in individuals with fibrous dysplasia / McCune-Albright syndrome

FD = fibrous dysplasia; GH = growth hormone; IGF-1 = insulin-like growth factor 1; SD = standard deviation

1. Hyperprolactinemia accompanies GH excess in approximately 80% of individuals with MAS. It usually only requires treatment if levels are very high and/or it is interfering with pubertal progression, menses, or sexual function [Salenave et al 2014].

2. The authors' practice is to add pegvisomant after reaching a maximal dose of somatostatin analogs.

3. Effective for treatment of modest elevations of GH and IGF-1 levels, with or without concomitant hyperprolactinemia.

4. Due to characteristic diffuse somatolactotroph hyperplasia of the pituitary, total hypophysectomy is required for successful surgical treatment [Vortmeyer et al 2012].

5. FD of the skull base is nearly universal in individuals with MAS-associated GH excess. There are reports of fatal skull base osteosarcomas arising after pituitary irradiation for treatment of MAS-associated GH excess [Liu et al 2011].

From: Fibrous Dysplasia / McCune-Albright Syndrome

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