Background: Hormone-secreting pituitary adenomas in children and adolescents are rare.
Methods: We report clinical course, management and outcome of 6 cases diagnoses in 2013-2019.
Results: Patients (3M, 3F), aged 9,5 16,5 years at referral. In them, 3 ACTH-secreting adenomas (ACTHA) and 3 prolactinomas (PROLA) were recognized. The family history for endocrine tumors was negative. In ACTHA, the patients' main complains were: growth retardation - 2, weight gain - 3, delay of puberty 1, striae 1, dysmenorrhoea 1. The children with PROLA had headache 3, primary amenorrhoea -1, visual impairment 3, delay of puberty 1. Additionally, 1 girl with ACTHA had polymorphic skin rush after sun exposure and glucose intolerance. In one boy with ACTHA, the clinical course of previous asthma had been improved. The boy with PROLA had unilateral exophtalmus and tachycardia. Hypercortisolism was confirmed in 3 patients by a high FUC levels. On MRI, 1 ACTHA presented as macroadenoma (13*17*26mm); two were microadenomas. In one MRI-negative case, the diagnosis was confirmed only after separate catheterization of cavernous sinuses with ACTH secretion on the right 40 times higher than on the left. All 3 ACTHA were treated by transsphenoidal surgery with neuronavigation. After surgery, one boy had transitory and one has permanent secondary adrenal insufficiency; one girl has central hypothyroidism. There were two macroPROLA- and 1 gigant PROLA.The prolactin initial levels were 2500- 138000 mU/l. Cabergoline was the first treatment in all patients. However, in the youngest girl PROLA was resistant to the weekly dose of 6 gr. and transsphenoidal surgery was done 2,5 years after conservative treatment with the normalization of prolactine level. Post-surgically, she is being treated with levothyroxine for central hypothyroidism. The gigant PROLA in a boy was also TSH-secreting; somatostatin analogue was added to the treatment with PRL and TSH suppression and progression of puberty. In the oldest girl, partial surgical tumor resection was performed as emergency due to an acute visual impairment, with further rapid decrease of PRL. However, she continues treatment with cabergoline. In this case, gonadotropin's deficiency was detected, also elevated levels of GH and IGF-1 were found. The patient may need additional treatment with somatostatin analogue, as well as sex steroid replacement.
Conclusion: Pituitary adenomas, even those hormonally active, represent a challenge for diagnosis and follow-up in children and adolescents. A good collaboration between pediatric endocrinologists, neurosurgeons and other specialists of the team can improve clinical outcomes of such patients.
19 - 21 Sep 2019
European Society for Paediatric Endocrinology