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病例报告:胸膜神经内分泌瘤转移到乳房,导致宫外库辛综合征
Aleksandra Zdrojowy-Wełna1, Marek Bolanowski1, Joanna Syrycka1,2
1Department and Clinic of Endocrinology and Internal Medicine, Wroclaw Medical University, Wroclaw, Poland.
Frontiers in oncology
|March 12, 2025
概括
从罕见的甲状腺神经内分泌瘤 (NETs) 中分泌的宫外 adrenocorticotropic 激素 (EAS) 可以导致严重的库辛综合征. 终身监测对于患有胸膜NET和EAS的患者至关重要,因为可能出现复发.
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 adrenocorticotropic 激素分泌 (EAS) 占库辛综合征的 10-18%.
- 胸膜神经内分泌瘤 (NETs) 是EAS的罕见原因,占病例的5-16%,可获得的数据有限.
- 胸膜NET患者通常比以前认为的年轻,高皮质醇症的严重程度与瘤大小无关.
研究的目的:
- 呈现一种罕见的异位上腺皮皮质激素分泌 (EAS) 复发的罕见病例,由胸腺神经内分泌瘤 (NET) 引起.
- 突出与导致库辛综合征的胸膜NET相关的诊断和治疗挑战.
- 强调核医学在诊断EAS方面的重要性以及长期患者监测的必要性.
主要方法:
- 一个34岁的妇女的病例报告,患有严重的高皮质醇症.
- 使用68Ga DOTA-TATE PET/CT 的诊断成像.
- 治疗涉及类固醇抑制剂 (基托可纳, metyrapone,奥西洛德罗斯塔特) 和手术瘤切除.
- 通过乳腺活检确认转移.
主要成果:
- 成功的初始治疗和在手术治疗胸膜 NET 后的高皮质醇症的解决.
- 由于乳腺转移而导致EAS的复发在初始治疗后5年,通过药物和手术成功管理.
- 首次治疗7年后的第二次复发,涉及局部中神经复发,用振荡器和胸腔镜手术治疗,获得良好的临床和生化反应.
- 患者仍然受到密切关注.
结论:
- 胸膜NET可以导致迅速发作,严重的高皮质醇症,即使是小瘤.
- 核医学成像,如68Ga DOTA-TATE PET/CT,对于确定EAS的来源至关重要.
- 终身监测对于被诊断为胸膜NET和EAS的患者至关重要,因为复发和转移的风险很高.
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