Department of Pediatric Surgery, Shizuoka Children's Hospital, Shizuoka, Japan.
J Pediatr Surg. 2012 Dec;47(12):2210-5. doi: 10.1016/j.jpedsurg.2012.09.010.
To evaluate the clinicopathological features that indicate relapse and suggest a new risk based therapeutic strategy for unilateral Favorable Histology Wilms Tumor (FH-WT).
MATERIALS & METHODS: Thirty-three patients with unilateral WT were treated in two institutions between 1986 and 2010. Twenty-eight patients with FH-WT received primary nephrectomy according to the National Wilms' Tumor Study (NWTS) or the Japanese Wilms' Tumor Study (JWiTS) protocol. Retrospective analyses of the non-relapsed group (n=23) and the relapsed group (n=5) compared age, gender, tumor laterality, tumor weight, initial tumor stage, known histological subtype, chemotherapy (2 or 3 drugs), and any irradiation delivered. Stages and histological subtypes of the tumors were re-evaluated according to the Japanese staging system.
Five of the twenty-eight tumors relapsed, and one patient died. The initial staging (P=0.029) and the histological subtype (P=0.003) were the only factors indicating relapse. Nine of the twenty-three tumors were histologically classified as blastemal predominant subtype (BPT-WT). Five relapsed.
According to the basic Japanese therapeutic strategy, all patients underwent a primary nephrectomy before chemotherapy. This study suggests that the histological subtype pre-treatment "BPT-WT" should be included as a strong indicator of poor prognosis. Such patients should be treated as a high-risk group.
评估提示复发的临床病理特征，并为单侧良好组织学型肾母细胞瘤（FH-WT）提出一种新的基于风险的治疗策略。
1986 年至 2010 年期间，两家机构共收治 33 例单侧 WT 患者。28 例 FH-WT 患者根据国家肾母细胞瘤研究（NWTS）或日本肾母细胞瘤研究（JWiTS）方案接受了原发肾切除术。对非复发组（n=23）和复发组（n=5）的回顾性分析比较了年龄、性别、肿瘤侧别、肿瘤重量、初始肿瘤分期、已知组织学亚型、化疗（2 种或 3 种药物）和任何照射情况。根据日本分期系统重新评估了肿瘤的分期和组织学亚型。
28 例肿瘤中有 5 例复发，1 例患者死亡。初始分期（P=0.029）和组织学亚型（P=0.003）是唯一提示复发的因素。23 例肿瘤中有 9 例组织学上分类为胚细胞瘤为主型（BPT-WT）。其中 5 例复发。
根据基本的日本治疗策略，所有患者在化疗前均接受了原发肾切除术。本研究提示，治疗前的组织学亚型“BPT-WT”应被视为预后不良的强烈指标。此类患者应被视为高危组。
