在儿童和青少年下垂体腺瘤的诊断和管理
Dominique Maiter1, Philippe Chanson2, Stefan Matei Constantinescu1
1Department of Endocrinology and Nutrition, UCLouvain Cliniques universitaires Saint Luc, 1200 Brussels, Belgium.
European journal of endocrinology
|October 7, 2024
概括
儿科垂体腺瘤 (PAs) 是罕见的,但存在独特的挑战. 这些瘤往往更大,更具攻击性,需要专门的多学科团队管理才能获得最佳结果.
科学领域:
- 儿科内分泌学 儿科内分泌学
- 神经瘤学神经瘤学
- 遗传学 是一个遗传学.
背景情况:
- 垂体腺瘤 (PAs),现在称为垂体神经内分泌瘤 (Pit-NETS),在儿童和青少年中很罕见,成人研究数据有限.
- 了解儿科PA是非常重要的,因为它们与成人瘤相比具有独特的特征.
研究的目的:
- 审查有关儿科和青少年垂体腺瘤的当前知识,涵盖流行病学,临床表现,诊断和治疗.
- 强调涉及儿科和成人专家的多学科团队 (MDT) 讨论对于管理这些罕见病例的重要性.
主要方法:
- 关于儿科和青少年垂体腺瘤的最新研究的文献综述.
- 对流行病学数据,临床特征,诊断方法和治疗方法的分析.
- 强调MDT在复杂儿科病例中的作用.
主要成果:
- 儿科PA通常更大,更具攻击性,并与像下垂体等质量效应相关.
- 荷尔蒙过分分泌是常见的,影响生长和青春期发育. 益生菌瘤是最常见的,其次是ACTH和GH分泌腺瘤.
- 临床非功能性腺瘤在16岁之前很罕见. 推进行基因检测以确定家族性倾向 (MEN1,AIP) 或马赛克突变 (麦昆-阿尔布赖特综合征).
结论:
- 儿科垂体腺瘤表现出独特的特征,需要专门的管理策略.
- 早期诊断,全面的遗传评估和MDT合作对于改善年轻患者的治疗结果至关重要.
- 需要进一步关注儿科特异性PA的研究,以完善治疗方案.
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