肾母细胞瘤的原发性肝转移——SIOP/GPOH研究报告

Primary hepatic metastases in nephroblastoma--a report of the SIOP/GPOH Study.

作者信息

Szavay Philipp, Luithle Tobias, Graf Norbert, Furtwängler Rhoikos, Fuchs Joerg

机构信息

Department of Pediatric Surgery, Children's Hospital, University of Tuebingen, 72076 Tuebingen, Germany.

出版信息

J Pediatr Surg. 2006 Jan;41(1):168-72; discussion 168-72. doi: 10.1016/j.jpedsurg.2005.10.021.

DOI:10.1016/j.jpedsurg.2005.10.021

PMID:16410128

Abstract

PURPOSE

Remarkable progress could be achieved in the treatment of nephroblastoma within the last decades. In all children with Wilms' tumor, 5-year overall survival rate reaches more than 90% in the SIOP/GPOH Study Group. Despite this fact, there is a small group of patients who have tumor lesions in the liver primarily representing a challenge in treatment. Data of this group are analyzed.

METHODS

To define survival and success of treatment in this group of patients, we reviewed the records of 29 of 1365 patients enrolled in the SIOP 93-01/GPOH Study and the SIOP 2001/GPOH Study between April 1, 1994, and September 30, 2004.

RESULTS

Median age at diagnosis was 10.61 years (range, 0.19-34.16 years). All patients but two underwent nephrectomy. Liver metastases were operated in 11 children at time of nephrectomy, in 4 patients secondarily, whereas in 11 patients, liver lesions were treated alone with chemotherapy and radiotherapy, respectively. In 3 children, no treatment could be initiated. Sixteen patients received radiotherapy additionally. Median follow-up was 64 months (range, 3-157 months). Eleven patients died in the course at a median of 13.07 months (range, 0.25-42 months) after initial diagnosis. These included 7 patients who never had surgery for their liver lesions and 4 patients who had incomplete and/or atypical resections of their metastatic liver lesions. All patients who underwent complete resection of hepatic metastases (n = 9) survived. Eight children survived with a nonsurgical treatment. Overall survival was below 60% in the whole group up to now.

CONCLUSIONS

Liver metastases are much less frequent than metastases to other sites. Our report suggests that Wilms' tumor complicated by metastases of the liver primarily has a less favorable outcome. Chemotherapy and radiotherapy play an important role in treatment. Radical surgery for nephrectomy as well as surgery of liver lesions cannot be overemphasized to prevent local and distant recurrence.

摘要

目的

在过去几十年中，肾母细胞瘤的治疗取得了显著进展。在SIOP/GPOH研究组中，所有患有威尔姆斯瘤的儿童5年总生存率达到90%以上。尽管如此，仍有一小部分患者主要在肝脏出现肿瘤病变，这对治疗构成了挑战。对该组患者的数据进行了分析。

方法

为了确定该组患者的生存率和治疗成功率，我们回顾了1994年4月1日至2004年9月30日期间参加SIOP 93 - 01/GPOH研究和SIOP 2001/GPOH研究的1365例患者中的29例的记录。

结果

诊断时的中位年龄为10.61岁（范围为0.19 - 34.16岁）。除两名患者外，所有患者均接受了肾切除术。11名儿童在肾切除时同时进行了肝转移灶手术，4例患者在肾切除术后进行了二次手术治疗肝转移灶，而11例患者的肝脏病变分别仅接受了化疗和放疗。3名儿童未开始治疗。16名患者还接受了放疗。中位随访时间为64个月（范围为3 - 157个月）。11例患者在初始诊断后的中位时间为13.07个月（范围为0.25 - 42个月）死亡。其中包括7例从未对其肝脏病变进行手术的患者和4例对其转移性肝脏病变进行了不完全和/或非典型切除的患者。所有接受肝转移灶完全切除的患者（n = 9）均存活。8名儿童通过非手术治疗存活。到目前为止，整个组的总生存率低于60%。