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诊断时伴有转移的肾母细胞瘤患者的临床病理特征及预后

Clinicopathologic features and prognosis for Wilms' tumor patients with metastases at diagnosis.

作者信息

Breslow N E, Churchill G, Nesmith B, Thomas P R, Beckwith J B, Othersen H B, D'Angio G J

出版信息

Cancer. 1986 Dec 1;58(11):2501-11. doi: 10.1002/1097-0142(19861201)58:11<2501::aid-cncr2820581125>3.0.co;2-v.

DOI:10.1002/1097-0142(19861201)58:11<2501::aid-cncr2820581125>3.0.co;2-v
PMID:3021319
Abstract

Comparisons were made between 236 Wilms' tumor patients with metastasis to the lungs and/or liver at initial diagnosis who were registered on the National Wilms' Tumor Study (NWTS) during 1969 to 1983, and 1755 patients who did not have overt metastases at diagnosis. Patients with evidence of regional spread of disease beyond the kidney, especially if to the renal vein or lymph nodes, were much more likely to have overt metastases present at diagnosis than those with apparently localized disease. The presence of metastases was also correlated with age at diagnosis, ranging from 1% among infants younger than 1 year of age to 24% for those aged 6 years or older. The percentage of tumor deaths for patients with metastases at diagnosis (Stage IV) and a primary tumor of favorable histology (FH) declined from 29% at 2 years postdiagnosis on the first therapeutic trial (NWTS-1) to 9% for the most recent one (NWTS-3), and is now comparable to that for patients without metastases but with nonresectable local invasion at diagnosis (Stage III). The local extent of disease also influenced the survival outcome for Stage IV/FH patients. Survival was poor for those with anaplastic or sarcomatous (unfavorable) histology, regardless of local staging or trial. There was no difference in survival according to metastatic site (liver +/- lung vs. lung only) if present prior to treatment. By contrast, patients who developed liver metastases during or after treatment had an especially poor chance for survival as compared with those who developed lung deposits at those times.

摘要

对1969年至1983年期间登记在国家肾母细胞瘤研究(NWTS)中的236例初诊时已发生肺和/或肝转移的肾母细胞瘤患者,与1755例初诊时无明显转移的患者进行了比较。有疾病超出肾脏区域扩散证据的患者,尤其是扩散至肾静脉或淋巴结的患者,初诊时出现明显转移的可能性比那些明显局限于局部的患者要高得多。转移的存在也与诊断时的年龄相关,1岁以下婴儿中转移的发生率为1%,而6岁及以上儿童中这一比例为24%。诊断时伴有转移(IV期)且原发肿瘤组织学类型良好(FH)的患者,其肿瘤死亡率从首次治疗试验(NWTS - 1)诊断后2年时的29%降至最近一次试验(NWTS - 3)时的9%,目前与初诊时无转移但有不可切除的局部侵犯的患者(III期)相当。疾病的局部范围也影响IV期/FH患者的生存结果。无论局部分期或试验情况如何,间变性或肉瘤样(不良)组织学类型的患者生存率都很低。如果在治疗前就已存在转移,根据转移部位(肝±肺与仅肺转移)的生存率没有差异。相比之下,与在治疗期间或治疗后出现肺转移的患者相比,那些在治疗期间或治疗后出现肝转移的患者生存机会尤其渺茫。

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