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儿童囊性肾瘤、囊性部分分化型肾母细胞瘤和囊性威尔姆斯瘤:一个存在治疗困境的谱系。

Cystic nephroma, cystic partially differentiated nephroblastoma and cystic Wilms' tumor in children: a spectrum with therapeutic dilemmas.

作者信息

van den Hoek Joop, de Krijger Ronald, van de Ven Kees, Lequin Maarten, van den Heuvel-Eibrink Marry M

机构信息

Department of Pediatric Urology, Erasmus MC, Sophia Children's Hospital, Rotterdam, The Netherlands.

出版信息

Urol Int. 2009;82(1):65-70. doi: 10.1159/000176028. Epub 2009 Jan 20.

Abstract

BACKGROUND

Cystic renal tumors are a diagnostic and therapeutic challenge. Cystic nephroma (CN), cystic partially differentiated nephroblastoma (CPDN) and cystic Wilms' tumor (CWT) are a spectrum with CN at the benign end, CWT at the malignant end and CPDN in the intermediate position. CN and stage 1 CPDN are often treated with surgery alone. International Society of Pediatric Oncology (SIOP) protocols for Wilms' tumor (WT) advocate preoperative chemotherapy, which may be unnecessary and potentially harmful in CN and in stage 1 CPDN. There are difficulties in differentiating the three types using imaging techniques. Therefore, controversies exist regarding the optimal treatment.

METHODS

We describe 6 children, who each had a postoperative diagnosis of CN, CPDN or CWT, and we retrospectively evaluate the treatment strategies.

RESULTS

The three types cannot be differentiated using imaging techniques, although the presence of solid components in the tumor is indicative of WT.

CONCLUSIONS

Surgery as first-line therapy should be seriously considered in children who have a cystic renal tumor, but further collaborative studies are needed since the distinction line between CPDN and CWT is not always clear.

摘要

背景

囊性肾肿瘤在诊断和治疗方面具有挑战性。囊性肾瘤(CN)、囊性部分分化肾母细胞瘤(CPDN)和囊性威尔姆斯瘤(CWT)构成一个谱系,CN位于良性一端,CWT位于恶性一端,CPDN处于中间位置。CN和1期CPDN通常仅通过手术治疗。国际小儿肿瘤学会(SIOP)的威尔姆斯瘤(WT)治疗方案主张术前化疗,但这对于CN和1期CPDN可能不必要且有潜在危害。使用影像学技术区分这三种类型存在困难。因此,关于最佳治疗方案存在争议。

方法

我们描述了6名儿童,他们术后分别被诊断为CN、CPDN或CWT,并对治疗策略进行回顾性评估。

结果

尽管肿瘤中实性成分的存在提示为WT,但使用影像学技术无法区分这三种类型。

结论

对于患有囊性肾肿瘤的儿童,应认真考虑将手术作为一线治疗方法,但由于CPDN和CWT之间的区分界限并不总是清晰,因此需要进一步的合作研究。

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