Pediatric Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milano, Italy.
Int J Radiat Oncol Biol Phys. 2012 Jan 1;82(1):348-54. doi: 10.1016/j.ijrobp.2010.09.022. Epub 2010 Nov 13.
We analyzed whether the prognosis can differ among Wilms tumors (WT) labeled as Stage III according to currently adopted classification systems.
Patients with nonanaplastic Stage III WT consecutively registered in two Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) trials (CNR-92, TW-2003) were the subjects in the present analysis. The steady mainstay of therapy was primary nephrectomy, followed by three-drug chemotherapy with vincristine, dactinomycin, doxorubicin, and abdominal radiotherapy (RT).
Ninety-nine WT patients met the criteria for classification as Stage III according to a revised version of the National Wilms Tumor Study-3 staging system (51 patients in CNR-92, 48 patients in TW-2003). Regional lymph nodes (LN) were not biopsied in 16 patients. After a median follow-up of 66 months, the 4-year disease-free survival (DFS) and overall survival (OS) rates were 85% ± 4% and 92% ± 3%, respectively, for the whole group. For 38 children with positive LN, the 4-year DFS rate was 73% ± 7%, as opposed to 98% ± 2% for the 45 children with Stage III WT according to the other criteria but with negative biopsied LN (p = 0.001). The subgroup with the worst prognosis consisted of children more than 2 years old with positive LN (DFS 67% ± 8%). A delay between surgery and RT > 30 days had an adverse impact on the abdominal tumor relapse rate.
This study provides further evidence that Stage III tumors with LN metastases might be distinguished from WTs meeting the other criteria for classification as Stage III. The worse outcome of the former may warrant a prospective study on the effects of intensified therapy. A subclassification of Stage III tumors is discussed.
我们分析了根据目前采用的分类系统，被标记为 III 期的 Wilms 肿瘤 (WT) 的预后是否存在差异。
本分析的对象是连续登记在意大利儿科血液学和肿瘤学会 (AIEOP) 两项临床试验 (CNR-92、TW-2003) 中的非间变型 III 期 WT 患者。治疗的主要方法是根治性肾切除术，随后进行长春新碱、放线菌素 D、多柔比星联合化疗和腹部放疗 (RT)。
根据修订后的国家 Wilms 肿瘤研究-3 分期系统 (CNR-92 组 51 例，TW-2003 组 48 例)，99 例 WT 患者符合 III 期分类标准。16 例患者未对区域淋巴结 (LN) 进行活检。中位随访 66 个月后，全组患者的 4 年无病生存率 (DFS) 和总生存率 (OS) 分别为 85%±4%和 92%±3%。38 例 LN 阳性儿童的 4 年 DFS 率为 73%±7%，而 45 例 LN 阴性但符合其他 III 期标准的 WT 儿童的 4 年 DFS 率为 98%±2% (p=0.001)。预后最差的亚组是年龄大于 2 岁且 LN 阳性的儿童 (DFS 为 67%±8%)。手术和 RT 之间的时间间隔>30 天对腹部肿瘤复发率有不利影响。
本研究进一步证实，LN 转移的 III 期肿瘤可能与符合其他 III 期分类标准的 WT 不同。前者的预后较差可能需要前瞻性研究强化治疗的效果。我们讨论了 III 期肿瘤的亚分类。
