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非洲地区儿童转移性 Wilms 瘤(WT)的治疗结果:肝转移是否对生存有负面影响?

The results of treatment of children with metastatic Wilms tumours (WT) in an African setting: do liver metastases have a negative impact on survival?

机构信息

Department of Paediatric Surgery, Nelson R. Mandela School of Medicine, University of KwaZulu-Natal, Inkosi Albert Luthuli Central Hospital, Durban, South Africa.

出版信息

Pediatr Blood Cancer. 2012 Aug;59(2):391-4. doi: 10.1002/pbc.24080. Epub 2012 Feb 7.

Abstract

BACKGROUND

From Africa, where socio-economic circumstances differ from the developed world, there are no data regarding the influence of liver metastases on survival of children with Wilms tumour.

PROCEDURE

One hundred fifty new patients with WT were seen between 2002 and 2010, 45 (30%) had metastases at diagnosis. Seven patients had bilateral disease with additional visceral metastases. Nine patients who developed liver metastases during treatment were excluded. The site of metastases and the results of pretreatment biopsies were retrieved. Neo-adjuvant chemotherapy was combined with nutritional resuscitation, and aggressive supportive care. Post-operative treatment was determined by stage and histology.

RESULTS

Liver metastases were present in 19 (42%) patients but were the sole metastatic site in only 4 (9%). Overall survival at 5 years was 58.5%. Event Free Survival was 54%. Thirty-three (73%) had favourable histology, nine unfavourable and undetermined in three. No influence of histology on outcome was evident. Three patients had resection of persistent liver metastases. The pattern of metastatic disease had no influence on outcome. Despite aggressive supportive care two patients (4%) died within a week of presentation. Two patients died of chemotoxicity and two of complications following biopsy. Eight patients (17%) were lost to follow-up of whom five were on palliative treatment only.

CONCLUSIONS

In Africa liver metastases do not appear to worsen the prognosis of children with Stage IV WT. Despite the poor socio-economic circumstances survival is comparable to other countries.

摘要

背景

非洲的社会经济情况与发达国家不同,目前尚无关于肝转移对儿童肾母细胞瘤生存影响的数据。

方法

2002 年至 2010 年间,我们共诊治了 150 例新发病例的 WT 患儿,其中 45 例(30%)在诊断时已有转移。7 例患儿存在双侧疾病,且伴有其他内脏转移。9 例在治疗过程中发生肝转移的患儿被排除在外。我们检索了转移部位和预处理活检的结果。新辅助化疗联合营养复苏和积极的支持治疗。术后治疗根据分期和组织学确定。

结果

19 例(42%)患儿存在肝转移,但仅 4 例(9%)为单纯肝转移。5 年总生存率为 58.5%。无事件生存率为 54%。33 例(73%)组织学表现良好,9 例组织学表现不良,3 例未确定。组织学对结局无影响。3 例患儿接受了持续性肝转移灶的切除术。转移疾病的模式对结局无影响。尽管给予了积极的支持治疗,但仍有 2 例患儿(4%)在就诊后 1 周内死亡。2 例患儿因化疗毒性而死亡,2 例患儿因活检相关并发症而死亡。8 例患儿(17%)失访,其中 5 例仅接受姑息治疗。

结论

在非洲,肝转移似乎不会使 IV 期 WT 患儿的预后恶化。尽管社会经济情况较差,但生存率与其他国家相当。

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