Background: Paediatric pituitary adenomas are rare and mostly benign disorders which may secrete pituitary hormones. Prolactinomas account for half of all pituitary adenomas (PROLA), followed by non-secreting adenomas (2040%; incidentalomas), adrenocorticotropic-hormone secreting adenomas (1030%; ACTHA) and growth-hormone-secreting adenomas (515%; GHA).
Methods: In this single-centre retrospective analysis clinical, biochemical and radiological features of paediatric patients with pituitary adenomas diagnosed between 2000 and 2016 were extracted from the electronic patients chart and analysed.
Results: 22 patients with pituitary adenomas were identified: 12 PROLA (54%, aged 1116.6 years; 8 females, 4 males), 4 incidentalomas (18%, aged 10.916 years; 3 females, 1 male), 3 ACTHA (14%, aged 812 years; one female, two males) and 3 GHA (14%, aged 9.3-17.9 years; three males). Incidentalomas and ACTHA were all microadenomas in contrast to GHA which were giant adenomas with infiltrative growth (diameter 1.96.2 cm). PROLA consisted of seven macro- (diameter 1.43.6 cm) and five microadenomas (diameter 0.30.9 cm). PROLA presented with headaches (67%) and pubertal delay (67%). All macro-PROLA with prolactin concentrations >10.000 mU/l had at least one pituitary hormone deficiency. ACTHA presented with cushingoid features and headaches. GHA displayed tall stature, headaches and hypopituitarism (2/3). Nausea, headaches, precocious puberty, mental retardation and epilepsy occurred in incidentalomas. Cabergoline induced normoprolactinemia (8/11), reduced mean tumour volume by 80% and ameliorated clinical signs in PROLA. Operative tumour resection was performed in ACTHA. Symptoms improved, but all of them experienced secondary adrenal insufficiency and hypopituitarism (n=1). GHA were partially resected followed by treatment with lanreotide or pasireotide, with clinical improvement in all and reduction of GH secretion by 56%. However, somatostatin analogous failed to reduce tumour volume. Incidentalomas were observed, but lost to follow-up.
Conclusion: Prolactinomas are most common pituitary adenomas even in children. In contrast to ACTHA and GHA clinical signs are unspecific in PROLA and incidentalomas. Adolescents with headaches and pubertal delay should be investigated for PROLA and incidentalomas. Cabergoline is effective and well tolerated in PROLA by reducing clinical symptoms, prolactin concentrations and inducing tumour shrinkage. Complete tumour resection in ACTH-secreting adenomas ameliorates clinical signs, but may be complicated by secondary adrenal insufficiency. Somatostatin analogues improve clinical symptoms in GHA, but do not reduce tumour size. Incidentalomas may only require symptomatic treatment or observation.
27 - 29 Sep 2018
European Society for Paediatric Endocrinology