罗莎-多夫曼病涉及整个食道
Wen-Peng Huang1, Liming Li2, Jianbo Gao2
1Nuclear Medicine, Peking University First Hospital.
Revista espanola de enfermedades digestivas
|June 23, 2023
概括
本案例研究详细介绍了一个儿童的罗赛-多夫曼病 (RDD),其特征是有广泛的食道病变. 治疗涉及化疗,在这种罕见的疾病中显示出积极的结果.
科学领域:
- 儿科瘤学 儿科瘤学
- 皮肤病学 皮肤病学
- 胃肠病学 胃肠病学
背景情况:
- 罗莎-多夫曼病 (RDD) 是一种罕见的良性增殖性疾病,通常表现为淋巴腺病变和外节干扰.
- 虽然RDD通常影响头部和部,但广泛的食道干扰是非常罕见的,特别是在儿科病例中.
研究的目的:
- 报告一个独特的儿科罗莎伊-多尔夫曼病病例,有显著的食道和鼻参与.
- 突出广泛的RDD的诊断挑战和多学科治疗方法.
- 讨论临床表现,成像发现和治疗结果.
主要方法:
- 一个有RDD的6岁男孩的案例介绍.
- 诊断工作包括MRI,18F-FDG PET/CT和多次活检.
- 治疗最初涉及皮质类固醇,随后是VCR+Ara-c+Dex化疗.
主要成果:
- 确认RDD与广泛的鼻腔,淋巴结,皮肤和食道病变.
- 最初的皮质类固醇治疗导致了消化不良,需要进一步调查.
- 18F-FDG PET/CT证明了广泛的代谢活性RDD参与.
- 化疗疗方案显示了积极的治疗反应.
结论:
- 罗莎伊-多夫曼病可以表现为广泛和不寻常的器官参与,包括儿童的食道.
- 多模式成像和活检对于诊断广泛的RDD至关重要.
- 组合化疗为严重的儿科RDD病例提供了一个有前途的治疗选择.
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