在诊断时存在肝转移的 Wilms 瘤患者，其并非 IV 期 Wilms 瘤的独立不良预后因素：来自儿童肿瘤学组/国立 Wilms 瘤研究组的报告。
Hepatic metastasis at diagnosis in patients with Wilms tumor is not an independent adverse prognostic factor for stage IV Wilms tumor: a report from the Children's Oncology Group/National Wilms Tumor Study Group.
机构信息
Renal Tumors Committee Children's Oncology Group, University of Michigan, Ann Arbor, MI 48109-0245, USA.
出版信息
Ann Surg. 2009 Oct;250(4):642-8. doi: 10.1097/SLA.0b013e3181b76f20.
OBJECTIVE
To determine event free survival (EFS) of children with Wilms tumor (WT) and metastatic liver disease at diagnosis.
SUMMARY AND BACKGROUND DATA
We reviewed patients with stage IV Wilms tumor treated on National Wilms Tumor Study 4 and 5 to ascertain if they have a worse prognosis than other Stage IV disease.
METHODS
A total of 742 patients (pts) with stage IV disease were assessed for EFS (95% confidence interval [CI]) at 5 years after diagnosis. Cohorts included those who underwent resection of the liver lesions compared with those who received only chemotherapy and radiotherapy.
RESULTS
A total of 742 patients with stage IV Wilms tumor were enrolled on NWTS-4 and 5, 111 of who had liver metastases. Of these, 96 had favorable histology disease and are the focus of this analysis. Twenty-two patients had a primary liver resection (wedge resection, 18 and lobectomy, 4). After chemotherapy and/or radiation, 13 patients underwent liver resection (wedge resection, 7; lobectomy, 5; and trisegmentectomy, 1). Seventy-one patients (67%) did not undergo surgery for their liver disease. In 14 patients, the liver disease disappeared with chemotherapy only. Eighty-two patients received abdominal radiation. EFS for the patients with metastatic FH Wilms tumor was 75% (95% confidence interval [CI]: (71%, 78%), EFS by Stage IV category was: lung only 76% (95% CI: 72%, 80%) (513 patients); liver, not lung 76% (95% CI: 58%, 87%) (34 patients), liver and lung 70% (95% CI: 57%, 80%) (62 patients), and other sites 64% (95% CI: 42%, 79%) (25 patients). There were no significant differences among stage IV groups (P = 0.60). EFS (95% CI) for the patients with primary resection of the liver metastases (22 patients) was 86% (63%, 95%) compared with 68% (56%, 78%) (P = 0.09) for the 74 with no primary resection of liver metastases. There was no significant difference in EFS for patients with FH Wilms tumor treated with chemotherapy compared with that of patients treated with chemotherapy and radiation (P = 0.63). The EFS (95% CI) for each of the subsets was; no abdominal radiation: 64% (34%, 83%); abdominal radiation, no boost: 77% (55%, 89%); abdominal radiation, boost: 72% (58%, 82%) (P = 0.05).
CONCLUSION
Liver metastasis at diagnosis is not an adverse prognostic factor for stage IV metastatic FH WT.
目的
确定初诊时患有肝转移的 Wilms 瘤（WT）患儿的无事件生存（EFS）。
摘要和背景数据
我们回顾了接受国家 Wilms 瘤研究 4 和 5 治疗的 IV 期 Wilms 瘤患者，以确定他们的预后是否比其他 IV 期疾病更差。
方法
共有 742 名患有 IV 期疾病的患者接受了诊断后 5 年 EFS（95%置信区间[CI]）评估。队列包括接受肝病变切除术的患者与仅接受化疗和放疗的患者。
结果
共有 742 名患有 IV 期 Wilms 瘤的患者在 NWTS-4 和 5 上登记，其中 111 名患有肝转移。其中，96 名患者有良好的组织学疾病，是本分析的重点。22 名患者接受了原发性肝切除术（楔形切除术 18 例，肝叶切除术 4 例）。在化疗和/或放疗后，13 名患者接受了肝切除术（楔形切除术 7 例，肝叶切除术 5 例，三叶切除术 1 例）。71 名患者（67%）未因肝疾病接受手术。在 14 名患者中，肝疾病仅通过化疗消失。82 名患者接受腹部放疗。转移性 FH Wilms 瘤患者的 EFS 为 75%（95%CI：（71%，78%）），按 IV 期分类的 EFS 为：仅肺 76%（95%CI：72%，80%）（513 例）；肝，非肺 76%（95%CI：58%，87%）（34 例），肝和肺 70%（95%CI：57%，80%）（62 例），和其他部位 64%（95%CI：42%，79%）（25 例）。各 IV 期组之间无显著差异（P=0.60）。接受肝转移原发切除术的患者 EFS（95%CI）为 86%（63%，95%），而未行肝转移原发切除术的 74 名患者 EFS 为 68%（56%，78%）（P=0.09）。与接受化疗的 FH Wilms 瘤患者相比，接受化疗和放疗的患者 EFS 无显著差异（P=0.63）。每个亚组的 EFS（95%CI）分别为：无腹部放疗：64%（34%，83%）；腹部放疗，无加量：77%（55%，89%）；腹部放疗，加量：72%（58%，82%）（P=0.05）。
结论
初诊时的肝转移不是 IV 期转移性 FH WT 的不良预后因素。
Zotero 插件