早期发病的青少年鼻血管纤维瘤 (JNA):一个系统的审查
Matthew Newman1, Thomas Boi Vu Nguyen2, Tobial McHugh1
1Otolaryngology-Head and Neck Surgery Division, Department of Surgery, McMaster University Medical Centre, 3V1 Clinic, 1200 Main St. West, Hamilton, ON, L8N 3Z5, Canada.
概括
在10岁以下的患者中,早期发病的青少年鼻血管纤维瘤 (EOJNA) 呈现出明显的特征. 与典型的JNA病例相比,这种罕见的疾病可能会导致更晚期的疾病和更高的复发率.
科学领域:
- 耳鼻喉科 耳鼻喉科 耳鼻喉科
- 儿科瘤学 儿科瘤学
- 头部和部手术 头部和部手术
背景情况:
- 青少年鼻血管膜瘤 (JNA) 通常影响青少年男性,发病时间通常在13-22岁之间.
- 早期发病的JNA (EOJNA),定义为年龄<10岁,很少见,并呈现异常的生长模式.
- 由于疾病特征和治疗结果的潜在差异,了解EOJNA至关重要.
研究的目的:
- 系统地审查和识别医学文献中所有报告的早期JNA病例.
- 分析EOJNA患者队列中的特定疾病特征,包括表现,分期和治疗方式.
- 将EOJNA患者的发现与一般人群中JNA的确定的特征进行比较.
主要方法:
- 在Embase,Cochrane和MEDLINE数据库中进行了全面的文献搜索,寻找1996年至2021年2月报告EOJNA病例的研究.
- 用描述性统计数据提取和分析了临床人口统计数据,疾病严重程度 (拉德科夫斯基阶段) 和治疗结果.
- 结果与所有年龄组的JNA报告的平均值进行了比较.
主要成果:
- 在29项研究中共发现了34例EOJNA病例,其中绝大多数 (31/34) 是男性患者.
- 对EOJNA诊断的平均年龄为8.15岁,常见的症状包括鼻腔阻塞 (65.2%) 和表 (60.9%).
- EOJNA患者经常出现晚期疾病 (拉德科夫斯基II/III阶段:78.8%),并经历了30%的复发率,在EOJNA组内,阶段或治疗类型没有显著差异.
结论:
- EOJNA病例表现出明显的特征,经常呈现出更先进的疾病.
- 与一般的JNA人群相比,这个队列的复发率更高.
- 建议提高对这种潜在的攻击性JNA亚型的临床意识.
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