Jackman Suzanne, Diamond Frank
All Children's Hospital, St Petersburg, Florida, USA.
Pediatr Endocrinol Rev. 2013 Jul;10(4):450-9.
Scientific advances are revealing the complexity of pituitary development, which is controlled by multiple transcription factors and signaling molecules. Unregulated pituitary cell growth, resulting in pituitary adenoma, is usually sporadic and results from monoclonal expansion of a single mutated cell. However, some adenomas develop as part of a genetic syndrome. Prolactinoma is the most common hormonally active pituitary adenoma in children. The non-functioning (non-secreting) pituitary adenoma is the second most common and often stains positive for GH, PRL, and/or TSH. While Cushing disease, resulting from an ACTH-secreting adenoma, commonly manifests as weight gain with growth deceleration in children, GH excess causes gigantism with rapid, accelerated growth inappropriate for the height of the family. TSH secreting pituitary adenomas are rare, and biochemical analysis will show an elevated thyroxine level with a non-suppressed or high TSH. Though the natural history of pituitary incidentalomas in children is unknown, adult practice guidelines are established.
科学进展正在揭示垂体发育的复杂性,垂体发育受多种转录因子和信号分子控制。不受调控的垂体细胞生长会导致垂体腺瘤,这种情况通常是散发性的,由单个突变细胞的单克隆扩增引起。然而,一些腺瘤是作为遗传综合征的一部分而发生的。泌乳素瘤是儿童中最常见的具有激素活性的垂体腺瘤。无功能(非分泌性)垂体腺瘤是第二常见的,并且通常对生长激素(GH)、泌乳素(PRL)和/或促甲状腺激素(TSH)染色呈阳性。由分泌促肾上腺皮质激素(ACTH)的腺瘤引起的库欣病,在儿童中通常表现为体重增加伴生长减速,而生长激素过多则导致巨人症,生长迅速且加速,与家族身高不相称。分泌促甲状腺激素的垂体腺瘤很罕见,生化分析会显示甲状腺素水平升高且促甲状腺激素未被抑制或升高。虽然儿童垂体意外瘤的自然病程尚不清楚,但已制定了成人实践指南。