Introduction: Pituitary adenomas are very uncommon in children, representing 3% of all pediatric intracranial neoplasms, and approximately 5% of all pituitary adenomas. Secreting tumors predominate, and longer disease trajectories are expected due to the patient age, cumulatively resulting in a natural history and treatment paradigm that are highly complex and often debated. We describe a large, single-institution series of pediatric pituitary adenomas, with extensive long-term follow-up.
Methods: The study cohort was compiled by searching institutional pathology and operative reports using diagnosis and site codes for pituitary and sellar pathology, from 1956-2016. Patients over 18 at diagnosis were excluded; included patients underwent retrospective chart review.
Results: Thirty-nine surgically managed pediatric pituitary adenomas were identified; median ages at times of diagnosis and surgery were 15 and 16 years, respectively (ranges 8-18; 9-22). Biochemical and pathologic analyses diagnosed 15 prolactinomas (39%), 14 corticotropinomas (36%), 7 somatotropinomas (18%), and 4 non-secreting adenomas (10%); 4 secreting tumors were plurihormonal. All patients underwent transsphenoidal resection as the initial surgical treatment (100%), with a history of failed pharmacotherapy in 13 prolactinomas. Primary surgical cure was achieved in 18 (46%); 21 experienced recurrent/persistent disease, with secondary treatments including repeat surgery in 10 (26%), radiation in 14 (36%), pharmacotherapy in 11 (28%), and bilateral adrenalectomy in 3 (21% of ACTH-secreting tumors). At last follow-up (median 87 months, range 3-581), 9 remained with recurrent/persistent disease (23%), with 0 mortalities.
Conclusions: Pediatric pituitary adenomas are rare, diverse, and challenging tumors with complexities far beyond those encountered in the management of routine adult pituitary disease, including nuanced decision making, a technically demanding operative environment, high propensity for recurrence, and the childhood-specific risks of debilitating morbidity from endocrine complications after surgery, radiation, or both. Optimal treatment requires a high degree of individualization, and patients are most likely to benefit from consolidated, multidisciplinary care in highly experienced centers.
Patient Care: Pediatric pituitary adenoma is rare and poorly understood; our research raises awareness regarding the elevated risks associated with this disease, including increased recurrence rates the frequent need for serial multi-modality treatments, and emphasizes the importance of expert care.
Learning Objectives: 1. Understand general principles of pediatric pituitary adenoma diagnosis and treatment
2. Review long-term surgical outcomes, including the elevated risk of recurrence and possible requirement for multi-modality therapy
3. Discuss differences between pediatric and adult pituitary disease