Clinical Presentation, Management, and the Outcomes of Pituitary Adenomas in Children

Aashish Sethi; Mohammed Didi; Poonam Dharmraj; Renuka Ramakrishnan; Senthil Senniappan; Urmi Das; Shivaram Avula; Ajay Sinha; Conor Mallucci; Christina Daousi; Catherine Gilkes; Nicola Thorp; Joanne Blair

Introduction: Pituitary adenoma (PA) in childhood is a rare disease, accounting for 3 % of all intracranial paediatric neoplasm, and between 3 to 6% of all PA. There are only few large studies describing paediatric pituitary adenoma and even fewer studies with long-term outcome.

Methods: In this retrospective study, clinical, biochemical and radiological parameters and outcome of paediatric patients (<16 years of age at diagnosis) with PA diagnosed between 2000 and 2019 were analysed.

Results: 23 patients with PA were identified with median follow up of 3.27 years (range 0.6 to 8.43): 12 prolactinomas (52%, median age: 15 years, range 13-16 years; 12 females), 6 non-functioning pituitary adenomas (NFPA) (26%, median age: 16 years, range 12-16 years; 2 females) and 5 adrenocorticotropic hormone (ACTH) adenomas (22%, median age: 13.5 years, range 4-15 years; 2 females). Prolactinomas consisted of nine macro (diameter 11mm-35mm) and three microadenomas (diameter 4-10 mm). Patients with prolactinoma presented with menstrual irregularities (67%), headache (50%), galactorrhoea (41%), and weight gain (41%). Cabergoline was effective in 9 patients, 3 required surgical intervention and 1 patient had recurrence after surgery and required radiotherapy. ACTH adenomas presented with weight gain (median BMI: 2.96 SDS; range: 2.12 to 5.47 SDS), short stature and headache. Operative tumour resection was performed in ACTH adenomas; 2 had recurrence and required radiotherapy while 1 patient required bilateral adrenalectomy (post-surgery and radiotherapy). All patients with ACTH adenomas developed multiple pituitary hormone deficiency (MPHD) post-surgery. Weight gain, visual field defects and headache were the presenting features in NFPA. Four required surgical intervention; two had recurrence post-surgery and required radiotherapy. On latest follow-up; 13 (56%) patients were obese (median BMI 3.09 SDS; range: 2.05 to 3.79 SDS). Five patients with aggressive disease required surgery and radiotherapy and suffered from post treatment weight gain (median BMI: 2.12 to 2.25 SDS).

Conclusion: Prolactinomas are the most common PA in children. Adolescents with headaches and menstrual irregularities should be investigated for prolactinoma. Cabergoline is effective in patients with prolactinoma in alleviating clinical symptoms, reducing prolactin concentrations and inducing tumour shrinkage. ACTH adenomas lead to aggressive disease and complete tumour resection could ameliorate clinical signs but can be complicated by recurrence. Surgery should be considered as first-line treatment in ACTH adenomas and in NFPA with visual impairment. Obesity is an important sequel and active identification and treatment is necessary.

ESPE Abstracts

P1-102