由尼尔森综合征复杂的子宫外大腔ACTH分泌腺瘤:一个病例报告
Laure Cécilia1, Galy Camille2, Fidani Thibault3
1Department of Diabetes and Endocrinology, University Hospital of Montpellier, Montpellier, France.
Annales d'endocrinologie
|February 8, 2026
概括
宫外ACTH分泌的垂体腺瘤是库辛综合征的罕见原因. 这一案例凸显了诊断挑战和核成像在定位这些瘤的实用性,特别是在大鼻腔的一个.
科学领域:
- 内分泌学 在内分泌学.
- 在瘤学瘤学.
- 核医学是一种核医学.
背景情况:
- 宫外ACTH分泌的垂体腺瘤位于土角外,是非常罕见的库辛综合征的原因.
- 由于局部化难以确定,诊断具有挑战性,往往导致治疗延迟.
- 拉特克囊中的残余细胞可以导致这些不寻常的瘤.
研究的目的:
- 报告一个宫外ACTH分泌性垂体腺瘤的病例,该病例发生在大鼻腔.
- 为了说明在局部化这种罕见的瘤的诊断困难.
- 突出先进的核成像技术在诊断中的作用.
主要方法:
- 一名38岁的妇女怀疑患有依赖ACTH的库辛综合征,接受了广泛的检查.
- 最初的脑垂体MRI结果是不确定的; hypophysectomy没有缓解.
- 18F-FDG PET/CT和体静止素受体扫描对于病变定位至关重要.
主要成果:
- 生物学测试表明宫外ACTH分泌,但瘤最初仍然难以捉摸.
- 尽管进行了脑下垂体手术和医疗治疗,但患者的病情并没有得到改善.
- 18F-FDG PET/CT和Octreoscan®在上鼻腔中发现了一个高代谢性病变.
- 手术切除证实了异位皮质形腺瘤,导致ACTH水平降低.
结论:
- 上鼻腔外皮皮质瘤在这种情况下导致了依赖ACTH的库辛综合征.
- 这一案例强调了与宫外ACTH分泌的垂体腺瘤相关的诊断挑战.
- 核医学成像,包括PET/CT和体静止素受体扫描,对于识别这些罕见病变非常有价值.
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