Background: Pituitary adenomas are rare in children. Most of them are found in adolescents. Macroadenomas and secreting adenomas are the most common. They can be sporadic, familial, belong to tumor syndromes and be associated with distinct genetic defects.
Objective and hypotheses: Report phenotypic and genotypic characteristics of pituitary adenomas in children.
Method: Eight children with pituitary adenoma were identified in 20 years. All underwent clinical examination, paraclinical Assessment (hypophysiogramme, MRI ± CT, Ophthalmic bilan) and a genetic study (Menine, AIP, FIPA).
Results: Average age was 7±0.4 (611) years. Sex ratio F/G: 1.5. Tumor syndrome is constant with ophthalmological disorders in 70% and gigantism in one case. The causes are: prolactin adenoma: 6; Somatotropic adenoma: 2. The tumor lesion is large in all cases: mean tumor height: 26 mm±0.8 (1845) Endocrine evaluation revealed dissociated anterior pituitary insufficiency n: 100% (GH: 7, TSH: 8, ACTH: 5). The genetic investigation revealed NEM 1 n: 4. The search for the AIP gene and FIPA was negative for the others cases.
Conclusion: Pituitary adenomas are exceptional in pre pubertal period. They are in large majority, characterized by clinical and genetic diversity. Prolactin adenoma is the most frequently. They must be diagnosed and treated precociously because of their locoregional agressivity.
10 - 12 Sep 2016
European Society for Paediatric Endocrinology