Pituitary tumors in childhood.

PURPOSE OF REVIEW

This review focuses on recently published literature on pituitary tumors in children with special focus on craniopharyngioma and prolactinoma. Although most pituitary masses are benign neoplasms, they are associated with high morbidity and mortality related to their proximity to vital neurologic structures including the optic chiasm, hypophysis and hypothalamus, resulting in disruption of pituitary hormone secretion.

RECENT FINDINGS

The role of Wingless/β-beta catenin signaling and aryl hydrocarbon receptor-interacting protein mutations in pituitary tumor formation has been recognized. There is general agreement on optimal treatment of prolactinomas, although duration of medical therapy in children deserves more specific attention. Advances in imaging, medical, surgical and radiotherapy techniques have greatly increased survival rates for all pituitary tumors, but high recurrence rates after attempted complete resection and high progression rates after incomplete resection of craniopharyngioma remain troublesome. Long-term morbidity and late mortality of all pituitary tumors have become better appreciated with recent focus placed on severe obesity. A trend toward a more multidisciplinary approach for craniopharyngioma, including radiotherapy and chemotherapy, particularly when tumor burden involves the hypothalamus, continues.

SUMMARY

A vast amount of literature has been recently published, focusing on the management and long-term sequelae of all pituitary tumors in children. Craniopharyngioma remains the most challenging mass arising within sellar and parasellar areas, and optimal treatment continues to be hotly debated. Recognizing the rarity of pediatric pituitary tumors, the field would greatly benefit from more prospective multicenter trials, as well as from standardization of the assessment of preoperative function and post-treatment outcomes.