额外上腺皮上腺癌与多重内分泌瘤1型相关
Deirdre Green1,2, Kate Richards3, Brendan Doyle3
1Department of Endocrinology, Beaumont Hospital, Dublin, Ireland.
Endocrinology, diabetes & metabolism case reports
|November 28, 2024
概括
本病例报告详细介绍了第一例患有多重内分泌新陈代谢1型 (MEN-1) 综合征的患者的副腎上腺皮层癌 (ACC) 的病例. 它强调在有风险的个体中对MEN-1进行查,并对上腺病变进行警的随访.
科学领域:
- 内分泌学 在内分泌学.
- 在瘤学瘤学.
- 遗传学 是一个遗传学.
背景情况:
- 上皮层癌 (ACC) 是一种罕见的上皮层恶性瘤.
- 多重内分泌新陈代谢1型 (MEN-1) 综合征是一种与ACC相关的罕见遗传疾病.
- 宫外ACC及其与MEN-1相关的情况极为罕见.
研究的目的:
- 在患有 MEN-1 综合征的患者中报告第一次额外上腺 ACC 病例.
- 突出MEN-1查在上腺病变患者的重要性.
- 为了强调对MEN-1患者上腺病变的密切临床监测的必要性.
主要方法:
- 一个66岁的女性新诊断出糖尿病和原发性副甲状腺功能障碍症的案例介绍.
- 对库辛综合征的荷尔蒙评估和对上腺原体的评估.
- 图像研究包括CT扫描检测上腺,胰腺和肺病变.
- 切除右上腺损伤的手术切除和肺和胰腺损伤的活检.
- 组织病理学和遗传学分析以确认诊断并确定MEN-1综合征.
主要成果:
- 组织学证实了额外上腺癌 (韦斯评分5/9) 和肺部转移性癌症.
- 一个胰腺神经内分泌瘤 (胰岛素瘤) 被诊断出.
- 基因检测证实了MEN-1综合征的存在.
- 该患者出现了ACTH独立的库辛综合征和原发性副甲状腺功能障碍症.
结论:
- 在MEN-1综合征中,上腺病变具有显著的恶性潜力.
- 早期检测和手术干预上腺病变在MEN-1中至关重要.
- 该病例是首次报告与MEN-1综合征相关的额外上腺ACC病例.
- 详细的病史和家族病史对于识别有 MEN-1 风险的个体至关重要.
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