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不可切除的肾母细胞瘤的管理与结局。国家肾母细胞瘤研究-3报告。

Management and outcome of inoperable Wilms tumor. A report of National Wilms Tumor Study-3.

作者信息

Ritchey M L, Pringle K C, Breslow N E, Takashima J, Moksness J, Zuppan C W, Beckwith J B, Thomas P R, Kelalis P P

机构信息

Division of Pediatric Surgery, University of Texas, Houston.

出版信息

Ann Surg. 1994 Nov;220(5):683-90. doi: 10.1097/00000658-199411000-00013.

DOI:10.1097/00000658-199411000-00013
PMID:7979618
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1234458/
Abstract

METHODS

The authors reviewed 131 children enrolled in National Wilms Tumor Study-3 (NWTS-3) who received preoperative treatment for tumors unable to be resected at surgery or judged inoperable by imaging evaluation. Preoperative biopsies were performed on 103 patients. Patients were assigned a pretreatment stage: stage II (11 patients), stage III (39 patients), stage IV (66 patients), and unknown (15 patients). The chemotherapy regimen included dactinomycin and vincristine (81 patients), dactinomycin, vincristine, and doxorubicin (30 patients), dactinomycin, vincristine, doxorubicin, and cyclophosphamide (10 patients), and other (8 patients). Preoperative radiation therapy was started concurrently with chemotherapy (27 patients) or because of lack of response (14 patients). Two patients were given preoperative irradiation without chemotherapy.

RESULTS

Response to therapy was assessed after the first trial of chemotherapy. Partial responses were noted in 110 patients (85%), 3 had complete responses, 13 had no response or progression of disease, and 5 patients were not able to be evaluated. There were no significant differences in preoperative response to the different chemotherapy regimens. Median time interval from diagnosis to nephrectomy was 58.5 days. When compared with NWTS-3 patients not receiving preoperative treatment, survival was reduced for patients treated preoperatively (88% vs. 74%, respectively, 4-year survival), which was only partially explained by differences in stage distribution. Median duration of follow-up was 5.9 years. Lack of response to the preoperative treatment was associated with a poor prognosis. Eight children died before removal of the primary tumor. All eight had either progressive disease or no response to the preoperative treatment.

CONCLUSIONS

The use of preoperative treatment can facilitate subsequent surgical resection in selected patients with inoperable Wilms tumors. Although these very large tumors--judged unable to be resected--have a somewhat worse prognosis, nephrectomy was completed in 93% of patients after preoperative treatment. However, preoperative treatment will lead to less accurate surgical and pathologic staging, and undertreatment should be avoided in these high-risk patients.

摘要

方法

作者回顾了131名参加国家肾母细胞瘤研究-3(NWTS-3)的儿童,这些儿童因肿瘤在手术时无法切除或经影像学评估判断为不可切除而接受了术前治疗。103例患者进行了术前活检。患者被分配了一个预处理阶段:II期(11例患者)、III期(39例患者)、IV期(66例患者)和未知期(15例患者)。化疗方案包括放线菌素和长春新碱(81例患者)、放线菌素、长春新碱和阿霉素(30例患者)、放线菌素、长春新碱、阿霉素和环磷酰胺(10例患者)以及其他方案(8例患者)。术前放射治疗与化疗同时开始(27例患者)或因无反应而开始(14例患者)。2例患者在未进行化疗的情况下接受了术前照射。

结果

在首次化疗试验后评估治疗反应。110例患者(85%)出现部分反应,3例完全缓解,13例无反应或疾病进展,5例患者无法评估。不同化疗方案的术前反应无显著差异。从诊断到肾切除术的中位时间间隔为58.5天。与未接受术前治疗的NWTS-3患者相比,术前接受治疗的患者生存率降低(4年生存率分别为88%和74%),这仅部分由分期分布差异所解释。中位随访时间为5.9年。术前治疗无反应与预后不良相关。8名儿童在切除原发肿瘤前死亡。所有8名儿童均有疾病进展或对术前治疗无反应。

结论

术前治疗的应用可促进部分不可切除肾母细胞瘤患者的后续手术切除。尽管这些被判断为无法切除的非常大的肿瘤预后稍差,但93%的患者在术前治疗后完成了肾切除术。然而,术前治疗会导致手术和病理分期的准确性降低,应避免在这些高危患者中治疗不足。

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