Background: Paediatric pituitary adenomas comprise rare but challenging pathologies in children and adolescents related to their endocrine and neurological characteristics.
Objective and Hypotheses: We aimed to describe a case report of growth hormone (GH)-secreting pituitary adenoma apoplexy.
Method: a 11-year-old girl presented with fever, sudden headache with vomiting. She abruptly developed bilateral hemianopia. On suspicion of infectious meningitis, lumbar puncture and brain MRI were performed on admission.
Results: The physical exam was normal, without neurological abnormalities except for visual disturbances. Auxological parameters were above 95th percentile (height 2.25 SDS, weight 1.85 SDS). Biochemistry laboratory and cerebrospinal fluid analysis were normal. Brain MRI showed a 33 mm-sellar and suprasellar mass with pituitary stalk and optic chiasm dislocation and third ventricle compression; it has heterogeneous signal features and contrast enhancement, highly suggestive of hemorrhagic transformation of a pre-existing pituitary adenoma. The endocrine investigations demonstrated central hypothyroidism, hypocortisolism, GH deficiency and diabetes insipidus. The lesion was surgically removed with a transphenoidal approach. The histological examination and immunohistochemical staining were compatible with somatotroph GH-secreting adenomas diffuse necrosis and hemorrhage areas. In the postoperative period, multiple pituitary hormone replacement therapy (hydrocortisone, desmopressin, estrogen and L-thyroxine) was started with good response. GH substitution was started 7 months later.
Conclusion: Pituitary apoplexy in children and adolescents is a rare entity that requires rapid and adequate treatment to prevent a life-threatening situation. Pituitary failure may develop with the effect of adenoma itself or following surgical excision. Clinical and laboratory signals may be suggestive and MRI neuroimaging is fundamental for diagnosis.
19 - 21 Sep 2019
European Society for Paediatric Endocrinology