[三例DENYS-DRASH综合征与纳多布拉斯托马的情况]
Mimu Ishikawa1,2, Naoya Tomomasa1,2, Shutaro Yamamoto1,2
1Division of Pediatic Urology, Department of Surgical Specialties, National Center for Child Health and Development.
概括
丹尼斯-德拉什综合征 (DDS) 患者可能会患上淋巴细胞瘤,这是恶性瘤的前体. 提前切除性腺非常重要,但由于脏疾病和威尔姆斯瘤风险,时间是复杂的.
科学领域:
- 儿科内分泌学 儿科内分泌学
- 儿科脏病学 儿科脏病学
- 儿科瘤学 儿科瘤学
背景情况:
- 丹尼斯-德拉什综合征 (DDS) 呈现出脏病,性发育障碍和威尔姆斯瘤.
- 阴道瘤在DDS中是一个显著的风险,需要去除阴道.
- 在DDS中进行淋巴切除术的最佳时间尚不清楚,这构成了临床挑战.
研究的目的:
- 在患有丹尼斯-德拉什综合征的患者中报告三例淋巴细胞母细胞瘤.
- 为了突出在DDS背景下切除的生殖腺中发生的淋巴细胞瘤.
- 讨论在DDS中确定淋巴切除术最佳时间的复杂性.
主要方法:
- 病例系列报道了三名被诊断患有丹尼斯-德拉什综合征的儿科患者.
- 对临床病史的审查,包括启动腹腔透析,切除术,移植和淋巴切除术.
- 切除性腺的病理检查,以确定性腺母细胞瘤和相关的病理.
主要成果:
- 淋巴细胞瘤是在所有三名DDS患者的切除性腺中发现的.
- 一名年龄在6岁1个月的患者出现了淋巴细胞瘤和淋巴细胞瘤,这是最年轻的病例之一.
- 这些发现强调了淋巴细胞瘤作为DDS早期事件的存在,即使有先前存在的脏病和威尔姆斯瘤的担忧.
结论:
- 淋巴细胞瘤是侵袭性淋巴腺瘤的前体,在DDS患者中需要及时切除.
- 在DDS中进行淋巴切除术的最佳时间是复杂的,受病进展和威尔姆斯瘤管理的影响.
- 这些病例强调了对患有丹尼斯-德拉什综合征的个体发生性腺瘤的警监测和手术干预的迫切需要.
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