Background. Xp11.2 translocation renal cell carcinoma, a rare malignancy, has a higher prevalence in children than in adults. It is relatively indolent in children but manifests with an aggressive course in adults. Clinical characteristics and prognostic studies for adult patients are scarce due to its rarity. Methods. This retrospective single-center study consecutively enrolled 24 newly diagnosed Xp11.2 translocation RCC adult patients. Clinical presentations were recorded, and baseline laboratory results and follow-up data were collected. Possible risk factors for progression-free survival and overall survival were first scanned with chi-square tests and -tests to compare patients who suffered from progression or death with who did not. Multivariate Cox regression was further utilized to identify independent risk factors. Results. Twenty-four adult patients (median age 32, range 16-73), with a male-to-female ratio of 1 : 1, was included from April 2010 to March 2020. After follow-up for 35.7 months (+/- months), seven patients died. With univariate analysis, higher C-reactive protein-to-albumin (CRP/Alb) ratio (), higher baseline fibrinogen (), and presence of distant metastasis () were associated with progression of the disease; higher preoperative fibrinogen () and distant metastasis () were associated with death. With multivariate Cox regression, only baseline fibrinogen level () was identified as an independent risk factor for progression-free survival; meanwhile, fibrinogen level () and distant metastasis () were identified as independent risk factors for survival. Conclusions. Overall, relatively high CRP/Alb ratios, fibrinogen, and distant metastasis were associated with a poor prognosis of Xp11.2 tRCC adult patients; among them, only baseline fibrinogen levels independently predicted the progression of Xp11.2 tRCC; thus, it may help to identify patients with worse progression or death risk.

中文翻译：

---

Xp11.2 易位肾细胞癌：临床特征和潜在的预后预测因子

背景。Xp11.2易位性肾细胞癌是一种罕见的恶性肿瘤，在儿童中的患病率高于成人。它在儿童中相对惰性，但在成人中表现为侵略性的过程。由于其罕见，成人患者的临床特征和预后研究很少。方法。这项回顾性单中心研究连续招募了 24 名新诊断的 Xp11.2 易位 RCC 成人患者。记录临床表现，收集基线实验室结果和随访数据。首先用卡方检验扫描无进展生存期和总生存期的可能风险因素，然后-测试以比较患有进展或死亡的患者与未患有进展或死亡的患者。进一步利用多变量 Cox 回归来识别独立的风险因素。结果。2010 年 4 月至 2020 年 3 月纳入 24 名成年患者（中位年龄 32 岁，范围 16-73 岁），男女比例为 1:1。随访 35.7 个月（+/- 个月） )，七名患者死亡。通过单变量分析，更高的 C 反应蛋白与白蛋白 (CRP/Alb) 比值 ()，较高的基线纤维蛋白原 (），以及远处转移的存在（)与疾病进展有关；较高的术前纤维蛋白原（)和远处转移 ()与死亡有关。使用多变量 Cox 回归，只有基线纤维蛋白原水平 ()被确定为无进展生存期的独立危险因素；同时，纤维蛋白原水平（)和远处转移 ()被确定为生存的独立危险因素。结论。总体而言，相对较高的 CRP/Alb 比率、纤维蛋白原性和远处转移与 Xp11.2 tRCC 成人患者的不良预后相关；其中，只有基线纤维蛋白原水平独立预测 Xp11.2 tRCC 的进展；因此，它可能有助于识别进展或死亡风险更严重的患者。