分泌ACTH的胰腺神经内分泌瘤:一个病例系列
Taymeyah Al-Toubah1, Eleonora Pelle1, Julie Hallanger-Johnson2
1Department of GI Oncology, H. Lee Moffitt Cancer Center & Research Institute, Tampa, Florida, USA.
Journal of neuroendocrinology
|September 9, 2023
概括
在胰腺神经内分泌新生瘤 (NENs) 中异位库辛综合征 (CS) 是罕见的,但具有攻击性,导致高死亡率. 早期诊断和双边上腺切除术等干预措施对于管理这种严重疾病至关重要.
科学领域:
- 内分泌学 在内分泌学.
- 在瘤学瘤学.
- 手术瘤学手术瘤学
背景情况:
- 宫外丘辛综合征 (CS) 是胰腺神经内分泌新生瘤 (NENs) 的罕见并发症.
- 与NEN相关的CS呈现出显著的发病率和死亡率.
- 早期识别和管理对于患者的治疗结果至关重要.
研究的目的:
- 为了研究患者的特征,治疗和结果,外宫CS由于胰腺NENs.
- 介绍ACTH分泌转移性胰腺NEN的最大的机构病例系列.
主要方法:
- 莫菲特癌症中心的胰腺NEN数据库的回顾性审查 (1/2008-4/2022).
- 识别和提取关于瘤特征,临床表现,治疗和生存的数据.
- 对13例已确定的子宫外CS病例的分析.
主要成果:
- 在胰腺NEN中发现了13例外宫CS病例,平均年龄为42岁.
- 大多数患者 (12/13) 呈现转移性疾病;瘤分化良好,其中两个转变为差分化.
- 癌症诊断后的平均整体存活期为56个月,CS诊断后的18个月;双边上腺切除术用于CS控制.
结论:
- 在胰腺NEN中异胎性CS是一种与侵袭性疾病相关的高度病态状况.
- 双边上腺切除术是一种可行的选择,可以控制严重的CS.
- 这项研究代表了ACTH分泌转移性胰腺NEN的最大案例系列.
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