Pituitary adenomas are rare in children and young people under the age of 19 (hereafter referred to as CYP) but they pose some different diagnostic and management challenges in this age group than in adults. These rare neoplasms can disrupt maturational, visual, intellectual and developmental processes and, in CYP, they tend to have more occult presentation, aggressive behaviour and are more likely to have a genetic basis than in adults. Through standardized AGREE II methodology, literature review and Delphi consensus, a multidisciplinary expert group developed 74 pragmatic management recommendations aimed at optimizing care for CYP in the first-ever comprehensive consensus guideline to cover the care of CYP with pituitary adenoma. Part 2 of this consensus guideline details 57 recommendations for paediatric patients with prolactinomas, Cushing disease, growth hormone excess causing gigantism and acromegaly, clinically non-functioning adenomas, and the rare TSHomas. Compared with adult patients with pituitary adenomas, we highlight that, in the CYP group, there is a greater proportion of functioning tumours, including macroprolactinomas, greater likelihood of underlying genetic disease, more corticotrophinomas in boys aged under 10 years than in girls and difficulty of peri-pubertal diagnosis of growth hormone excess. Collaboration with pituitary specialists caring for adult patients, as part of commissioned and centralized multidisciplinary teams, is key for optimizing management, transition and lifelong care and facilitates the collection of health-related quality of survival outcomes of novel medical, surgical and radiotherapeutic treatments, which are currently largely missing.
Sections
Consensus statementDiagnosis: clinical features.• Part 2: R1. Offer serum prolactin measurement in CYP presenting with one or more of the following signs and symptoms: delayed puberty; galactorrhoea; visual field loss; growth or pubertal arrest; or girls with menstrual disturbance (strong recommendation, moderate-quality evidence).High serum levels of prolactin inhibit gonadotrophin secretion via inhibition of the hypothalamic hormone kisspeptin 14 . Paediatric patients with hyperprolactinaemia might therefore present with delayed (>2 standard deviations (SD) later than mean population age for sex) or arrested puberty, growth failure or short stature, primary amenorrhoea, galactorrhoea, menstrual disturbance or secondary amenorrhoea (in post-menarcheal girls) 3,6 . Boys might present with gynaecomastia as a result of hypogonadism. Mass effects, occurring more commonly in boys than girls, include headache and visual field loss 15 . Obesity, gynaecomastia, constitutional delay in growth and puberty in boys, and menstrual disturbance in girls are common physiological variations that are very rarely caused by prolactinoma. However, the cost of measuring prolactin is offset by the benefits of an early diagnosis and timely treatment.Diagnosis: biochemical evaluation.• Part 2: R2. In CYP with signs or symptoms of hyperprolactinaemia, ...
