BSPED2018 Oral Communications Oral Communications 4 (8 abstracts)
1Kings College Hospital NHS Trust, London, UK; 2Guys and St. Thomas NHS Trust, London, UK.
Objective: To describe the clinical presentation, management and treatment outcomes of prolactinomas diagnosed in childhood and adolescence in a consecutive series.
Design and Methods: A retrospective review of medical records of patients with prolactinoma less than 20 years at diagnosis, referred to a tertiary paediatric endocrine service between 1996 and 2018.
Results: Twenty-three patients (14 females) were identified; median age at diagnosis 15.7 years (range 1319) and median follow up 36 months (range 2156 months); 13 patients had a macroprolactinoma. Pubertal disorder, galactorrhoea and headache were the commonest presenting symptoms. Although the difference was not statistically significant, there was a definite trend towards larger tumour size at presentation in males as compared to females (21 mm vs 9 mm; P=0.076). Seven patients (all macroprolactinomas) had associated pituitary hormone deficiencies at presentation. Co-existing growth hormone excess was present in one patient. The majority (82%) of patients demonstrated good clinical, biochemical and radiological response to dopamine agonist therapy (cabergoline). Six patients underwent surgical resection cabergoline unresponsive (3), pituitary apoplexy (2), and diagnostic confirmation (1). Patients requiring surgical intervention were invariably macroadenomas greater than 20 mm in diameter at presentation. Patients undergoing surgery had larger tumours (P=0.008) and higher serum prolactin concentration (P=0.046). Two of these six patients who underwent surgical resection also required Temezolomide and radiotherapy to achieve disease control. New anterior pituitary function deficits were infrequent after surgical resection (17%). One patient was known to be MEN1 positive. Of the remaining patients (9) tested, none were found to have an AIP or MEN1 mutation.
Conclusions: Prolactinomas are rare below the age of 20 years, occurring mainly during adolescence. Microprolactinoma predominantly occurs in girls and is very effectively treated with cabergoline. Macroprolactinoma occurs predominantly in boys, presents with mass effect and particularly if greater than 20 mm in size, may require multimodal therapy and are more likely to have hormone deficiencies. Due to rarity of these tumours in this age group, paediatric and adolescent patients benefit from being managed in shared/transitional care with the adult endocrine and neurosurgical teams.