Tarek Nidale, Said Rabih, Andersen Clark R, Suki Tina S, Foglesong Jessica, Herzog Cynthia E, Tannir Nizar M, Patel Shreyaskumar, Ratan Ravin, Ludwig Joseph A, Daw Najat C
Department of Pediatrics, the University of Texas MD Anderson Cancer Center, Houston, TX 77030, USA.
Department of Pediatrics and Adolescent Medicine, American University of Beirut Medical Center, Beirut 1107, Lebanon.
Cancers (Basel). 2020 Oct 11;12(10):2927. doi: 10.3390/cancers12102927.
Limited information exists on the clinical behavior of the Ewing sarcoma family of tumors (ESFT) of the kidney. We reviewed the records of 30 patients (aged 8-69 years) with ESFT of the kidney seen at our institution between 1990 and 2013. We analyzed the event-free survival (EFS) and overall survival (OS) for associations with patient demographics, disease group, tumor size, tumor thrombus, and treatment. Six patients (20%) had tumors confined to the kidney (Group I), seven (23.3%) had local tumor extension beyond the kidney (Group II), and 17 (56.7%) had distant metastasis at diagnosis (Group III). Twenty-five (83.3%) patients underwent radical (19 upfront, five delayed) or partial (one upfront) nephrectomy, 25 (83.3%) chemotherapy and four (13.3%) radiotherapy. The 4-year EFS and OS were 43% (95% CI, 26-61%) and 63% (95% CI, 46-81%), respectively. EFS and OS were significantly associated with disease group and chemotherapy ( < 0.039). The presence of tumor thrombus in renal vein and/or inferior vena cava was associated with worse EFS ( = 0.053). Patients with disease confined to the kidney treated with nephrectomy and adjuvant chemotherapy have favorable outcomes. Local tumor extension beyond the kidney, tumor thrombus, and distant metastasis are unfavorable factors that warrant intensification or novel approaches of therapy.
关于肾尤因肉瘤家族性肿瘤（ESFT）的临床行为，现有信息有限。我们回顾了1990年至2013年间在我们机构就诊的30例（年龄8 - 69岁）肾ESFT患者的记录。我们分析了无事件生存期（EFS）和总生存期（OS）与患者人口统计学、疾病分组、肿瘤大小、肿瘤血栓及治疗之间的关联。6例（20%）患者的肿瘤局限于肾脏（I组），7例（23.3%）有局部肿瘤延伸至肾脏以外（II组），17例（56.7%）在诊断时已有远处转移（III组）。25例（83.3%）患者接受了根治性（19例为初始治疗，5例为延迟治疗）或部分（1例为初始治疗）肾切除术，25例（83.3%）接受了化疗，4例（13.3%）接受了放疗。4年EFS和OS分别为43%（95%CI，26 - 61%）和63%（95%CI，46 - 81%）。EFS和OS与疾病分组及化疗显著相关（P < 0.039）。肾静脉和/或下腔静脉中存在肿瘤血栓与较差的EFS相关（P = 0.053）。接受肾切除术和辅助化疗且疾病局限于肾脏的患者预后良好。局部肿瘤延伸至肾脏以外、肿瘤血栓及远处转移是不利因素，需要强化治疗或采用新的治疗方法。
