Background: Gigantism is a condition characterized by excessive growth and height significantly above average; it is usually caused by a tumor on the pituitary gland. In some cases the condition can be passed on genetically through a mutated gene.
Objective and hypotheses: Describe clinical, hormonal, and morphological profile of seven children with gigantism. Mean age=14 years (916), age of onset of symptoms 12 years. Tall stature (50%), and brain tumor syndrome (50%). Discrete dysmorphic syndrome (n=1). Delayed puberty (n=4) a McCuneAlbright syndrome (n=1), no signs of MEN type 1.
Method: Laboratory studies used in the diagnosis: of gigantism: OGTT/GH and IGF1 of hypopituitarism: cortisol, ACTH, FT4, TSH, FSH, LH, E2, TESTO, PRL. Imaging studies include Magnetic resonance imaging (MRI): to image pituitary adenomas.
Results: Average of GH=110 μUI/ml and high IGF1. Hyperprolactinemia (n=2), gonadal failure (n=3), hypothyroidism (n=1 case), and normal cotisol level (n=7). Hypothalamicpituitary MRI: locally invasive pituitary adenoma (n=4) and aggressive (n=2). Treatment: surgery (n=6), second surgery (n=3), surgery+radiotherapy (n=4), surgery+radiotherapy+somatostatin analogs (AS) (n=3), surgery+AS (n=1 case), and AS (n=1).
Conclusion: Gigantism is a rare disease of the child. A monitoring of growth allows early diagnostic and early treatment; because the Treatment is difficult and requires a combination of several treatment arms. In addition there is no indication of AS before age=15years. The genetic study is required.
20 - 22 Sep 2014
European Society for Paediatric Endocrinology