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患有来自神经结骨瘤的儿科外宫ACTH综合征的挑战

Ruochen Che¹, Jianfeng Zhou², Chunlei Zhou³

¹Department of Nephrology, Children's Hospital of Nanjing Medical University, Nanjing, China.

Journal of pediatric endocrinology & metabolism : JPEM

|February 28, 2026

概括

在儿童中异卵性ACTH综合征 (EAS) 是罕见的,但一个病例与神经囊瘤有关. 像PET/CT这样的先进成像对于诊断这些具有挑战性的神经内分泌瘤至关重要.

科学领域:

儿科内分泌学儿科内分泌学
在瘤学瘤学.
核医学就是核医学.

背景情况:

宫外ACTH综合征 (EAS) 是一种罕见的内分泌疾病,特别是在儿童群体中.
圣甲骨瘤 (SCTs) 很少与EAS有关,这给诊断带来了挑战.
儿童库辛综合征需要对罕见的潜在原因进行彻底的调查.

研究的目的:

报告第一个由SCT内的神经内分泌瘤引起的EAS青少年病例.
审查有关诊断困难和SCT相关EAS的管理的文献.
强调高级成像在识别隐藏的神经内分泌成分方面的作用.

主要方法:

一名13岁的女性患有高皮质醇症的症状,接受了生物化学测试和成像.
使用18F-FDG PET/CT和68Ga-DOTA-NOC PET/CT来描述一个前圣体质量.
手术切除和组织病理学分析证实了瘤内的神经内分泌瘤.

主要成果:

生物化学测试证实了ACTH依赖的高皮质醇症;垂体成像是负的.
68Ga-DOTA-NOC PET/CT检测显示,在神经前部质量中,体内静止素受体表达强烈.
组织病理学发现了一个成熟的瘤与ACTH和SSTR2阳性神经内分泌瘤 (NET G1).
观察到手术后激素水平的正常化和症状的消失.

结论:

这个病例代表了第一个与SCT内的神经内分泌瘤相关的青少年EAS.
补充PET/CT成像,评估FDG代谢和SSTR表达,对于定位隐藏的神经内分泌瘤至关重要.
成功的管理依赖于完整的瘤切除和多学科方法,改善儿科EAS的结果.

关键词:

异胎ACTH综合征是什么意思低血糖症 (hypokalemia) 是一种疾病,神经内分泌瘤是一个神经内分泌瘤.小儿科库辛综合征库辛综合征这是一种神圣的瘤 (sacrococcygeal teratoma).

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