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Clinical characteristics of Xp11.2 translocation renal cell carcinoma

Xp11.2易位肾细胞癌的临床特征

基本信息

批准号：
23791740
负责人：
NUMAKURA Kazuyuki
金额：
$ 2.58万
依托单位：
Akita University
依托单位国家：
日本
项目类别：
Grant-in-Aid for Young Scientists (B)
财政年份：
2011
资助国家：
日本
起止时间：
2011 至 2012
项目状态：
已结题

来源：
https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-23791740/
关键词：
腫瘍学 Xp11.2転座腎癌腎細胞癌 Xp1.2転座 Xp11.2転座

项目摘要

Introduction. Xp11.2 translocation renal cell carcinomas (RCCs) have been reported to be common in children and relatively rare in adults. In this retrospective multicenter study, we aimed to characterize Xp11.2 translocation RCCs in adult Japanese patients.Methods. Immunohistochemical detection of TFE3 in paraffin-embedded samples obtained from 963 adult Japanese RCC patients, who had undergone radical nephrectomy or tumor biopsy, was performed using the polyclonal peptide antibody sc-5958. The Xp11.2 translocation was confirmed by TFE3 split fluorescence in situ hybridization (FISH) analysis in patients with positive TFE3 immunohistochemistry. Clinical and pathological characteristics of Xp11.2 translocation RCCs were analyzed.Results. Immunohistochemical analysis identified 29 patient samples (17 males and 12 females) positive for TFE3 (3.0%) staining. Five of these samples were positive by FISH analysis. The median age of TFE3-positive patients was 46 years (range, 22.73 years). Th … More e mean tumor size was 7.1 cm. Fourteen (48.3%) patients were classified as stage pT3 or pT4, and 12 patients (41.4%) diagnosed as N+ and/or M+. The mean follow-up period was 32.5 months. De novo metastatic lesions were found in 2 patients after nephrectomy. Among 14 patients with metastatic disease, cytokine therapy, tyrosine kinase inhibitors (TKI), or immunochemotherapy was administered to 8, 4, and 2 patients, respectively.Objective responses (OR) were achieved in 2 (50%) patients treated with TKI, 2 (100%) patients treated with immunochemotherapy, and none in patients treated with cytokines. The median overall survival (OS) of all patients and patients with metastatic disease were 34.9 months and 22.0 months, respectively. Conclusion. We observed a 3.0% incidence of Xp11.2 translocation RCCs in adult Japanese patients, which was similar to the rate reported previously. Half the patients presented with advance stage disease at diagnosis, and the prognosis seems to be worse than that for clear cell carcinoma. TKI or immunochemotherapy resulted in ORs in 50% of the patients, while cytokine therapy was ineffective. Further molecular characterization of these tumors is required to identify more efficacious treatment modalities. Less

导论.据报道，Xp11.2易位肾细胞癌（RCC）在儿童中很常见，在成人中相对罕见。在这项回顾性多中心研究中，我们的目的是描述日本成年患者中Xp11.2易位RCC的特征。方法。使用多克隆肽抗体SC-5958进行了TFE 3的免疫组织化学检测，石蜡包埋的样本来自963例日本成人RCC患者，他们接受了根治性肾切除术或肿瘤活检。在TFE 3免疫组化阳性的患者中，通过TFE 3裂解荧光原位杂交（FISH）分析证实了Xp11.2易位。分析Xp 11. 2易位型肾细胞癌的临床及病理特点。免疫组织化学分析鉴定出29个患者样品（17个男性和12个女性）为TFE 3染色阳性（3.0%）。其中5份样本经FISH分析呈阳性。TFE 3阳性患者的中位年龄为46岁（范围：22.73岁）。日 ...更多信息平均肿瘤大小为7.1厘米。14例（48.3%）患者被分类为pT 3或pT 4期，12例（41.4%）被诊断为N+和/或M+。平均随访时间为32.5个月。2例肾切除术后发现原发性转移灶。在14例转移性疾病患者中，细胞因子治疗8例，酪氨酸激酶抑制剂（TKI）治疗4例，免疫化疗2例，TKI治疗2例（50%），免疫化疗2例（100%），细胞因子治疗0例。所有患者和转移性疾病患者的中位总生存期（OS）分别为34.9个月和22.0个月。结论我们观察到日本成人患者Xp11.2易位RCC的发生率为3.0%，与以前报道的发生率相似。一半的患者在诊断时表现为晚期疾病，预后似乎比透明细胞癌更差。TKI或免疫化疗在50%的患者中导致OR，而细胞因子治疗无效。这些肿瘤的进一步分子表征需要确定更有效的治疗方式。少