儿童骶前肿块：表现、病因和恶性风险。
Presacral masses in children: presentation, aetiology and risk of malignancy.
机构信息
Department of Surgery, Academic Medical Center, Amsterdam, the Netherlands.
出版信息
Colorectal Dis. 2011 Aug;13(8):930-4. doi: 10.1111/j.1463-1318.2010.02312.x. Epub 2010 May 17.
AIM
The risk of malignant changes in presacral tumours in children was investigated in relation to age at diagnosis, type of presentation and origin of the tumour.
METHOD
A retrospective review was carried out in 17 patients surgically treated for congenital presacral masses over a 22-year period.
RESULTS
Constipation was the main symptom in 14 (82%) of 17 patients. The lesions were evident on digital examination in 14 patients. Mature teratoma (n = 9, 64%) was the most common lesion, including three malignancies. Currarino syndrome was diagnosed in 10 (71%) patients. Two unclassified variant HLXB9 gene mutations were found in five (29%) patients who underwent genetic testing.
CONCLUSION
Congenital presacral tumours in children were mostly mature teratomas, either as sacrococcygeal teratomas or as part of the Currarino syndrome. The risk of malignancy in patients older than 1 year necessitates early surgical resection.
目的
研究儿童骶前肿瘤的恶性变化风险与诊断时的年龄、表现类型和肿瘤起源之间的关系。
方法
对 17 例在 22 年期间接受先天性骶前肿块手术治疗的患者进行回顾性分析。
结果
17 例患者中，14 例（82%）主要症状为便秘。14 例患者在数字检查中发现病变。成熟畸胎瘤（n=9，64%）是最常见的病变，其中包括 3 例恶性肿瘤。诊断出 Currarino 综合征 10 例（71%）。对 5 例（29%）接受基因检测的患者进行基因检测，发现了两种未分类的 HLXB9 基因突变。
结论
儿童先天性骶前肿瘤多为成熟畸胎瘤，既有骶尾部畸胎瘤，也有 Currarino 综合征的一部分。1 岁以上患者的恶性肿瘤风险需要早期手术切除。
Zotero 插件