Department of Pediatric Endocrinology, Dartmouth Medical School, Children's Hospital at Dartmouth, Lebanon, New Hampshire, USA.
Curr Opin Endocrinol Diabetes Obes. 2012 Feb;19(1):63-7. doi: 10.1097/MED.0b013e32834ed6b9.
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.
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.
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.
本篇综述重点关注了近期发表的儿童垂体瘤文献，尤其聚焦颅咽管瘤和泌乳素瘤。虽然大多数垂体肿块为良性肿瘤，但由于其靠近视神经交叉、垂体和下丘脑等重要神经结构，导致垂体激素分泌紊乱，与较高的发病率和死亡率相关。
已认识到 Wingless/β-连环蛋白信号和芳香烃受体相互作用蛋白突变在垂体瘤形成中的作用。虽然对于泌乳素瘤的最佳治疗方法已达成共识，但儿童的药物治疗持续时间需要给予更多关注。影像学、药物、手术和放疗技术的进步极大地提高了所有垂体瘤的生存率，但颅咽管瘤在试图完全切除后复发率高，在不完全切除后进展率高，仍然是棘手的问题。由于最近关注严重肥胖问题，所有垂体瘤的长期发病率和晚期死亡率得到了更好的认识。对于颅咽管瘤，倾向于采用更具多学科性的方法，包括放疗和化疗，尤其是当肿瘤负荷涉及下丘脑时。
大量文献最近集中于儿童所有垂体瘤的管理和长期后果。颅咽管瘤仍然是鞍区和鞍旁区最具挑战性的肿块，最佳治疗方法仍存在激烈争议。鉴于儿童垂体瘤罕见，该领域将大大受益于更多前瞻性多中心试验，以及标准化术前功能评估和治疗后结果的评估。
