图纳综合征和Y染色体马赛克症患者的淋巴腺瘤:一项回顾性多站点研究
Tazim Dowlut-McElroy1, Jessica R Long2, Allison C Mayhew3
1Pediatric and Adolescent Gynecology, Department of Surgery, Children's Mercy Hospital, University of Missouri-Kansas City School of Medicine, Kansas City, Missouri.
患有特纳综合征和Y染色体马赛克 (TS+Y) 的个体有24.6%的淋巴腺瘤患病率,恶性瘤的风险较低. 对那些推迟淋巴切除术的患者的临床监测缺乏一致性.
科学领域:
- 内分泌学 在内分泌学.
- 遗传学 遗传学 是一个
- 儿科瘤学 儿科瘤学
背景情况:
- 带有Y染色体马赛克的特纳综合征 (TS+Y) 在生殖腺发育和瘤风险方面存在独特的挑战.
- 预防性淋巴切除术经常被考虑,但对那些推迟该程序的人的临床监测实践并不成熟.
研究的目的:
- 为了确定TS+Y个体中生殖细胞瘤的患病率.
- 评估推迟预防性淋巴切除术患者的临床监测策略.
主要方法:
- 来自北美儿童医院的57名TS+Y患者 (0-30岁) 的回顾性图表审查.
- 医疗记录的分析,包括淋巴切除术状态,淋巴腺瘤的存在以及临床后续计划.
主要成果:
- 在队列中观察到 24.6% 的淋巴腺瘤患病率.
- 淋巴细胞瘤 (29.8%) 和乳腺瘤 (4.3%) 是最常见的类型.
- 包括超声波和瘤标志物在内的监测实践在没有经历淋巴切除术的参与者中缺乏标准化.
结论:
- 患有TS+Y的个体有明显的淋巴腺瘤患病率,尽管恶性转变的风险很低 (3.5%).
- 增长激素治疗没有预测淋巴腺瘤的存在.
- 进一步的细胞遗传研究是必要的,以阐明在这个人群中促进淋巴腺瘤发展的因素.
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