目的:报告单个大体积中心的Xp11.2易位/转录因子E3(TFE3)基因融合肾细胞癌(Xp11.2易位RCCs)的临床病理特征和中长期肿瘤学结果。
方法:回顾性分析46例诊断为Xp11.2易位RCC并接受手术治疗的患者的临床和随访资料。
结果:从4218例肾脏肿瘤患者中确定了46例Xp11.2易位RCC患者,这些患者从1月开始在我们的中心接受了手术2014年4月。2020年。我们中心Xp11.2易位RCCs的发生率为1.09%。在30.5个月的中位随访期间,4例患者死于该疾病。总中位总生存期和癌症特异性生存期分别为30.0个月和24.0个月。分别。1年,3年和5年OS率为97.4%,88.8%,和88.8%,分别。在多变量分析中,诊断时显示症状(p=0.019),淋巴结转移(p=0.002)和远端转移(p=0.020)被确定为不良预后的危险因素。
结论:Xp11.2易位RCC是一种肾细胞癌,发病率相对较低,预后各异。早期Xp11.2易位RCC与大多数典型RCC的预后相似,但晚期Xp11.2易位RCCs可导致不良的肿瘤学结局。
OBJECTIVE: To report the clinicopathological features and mid- to long-term oncologic results of Xp11.2 translocation/transcription factor E3 (TFE3) gene fusion renal cell carcinomas (Xp11.2 translocation RCCs) in a single large-volume centrecentre.
METHODS: Clinical and follow-up data of 46 patients who were diagnosed with Xp11.2 translocation RCC and underwentunderwent surgical intervention were retrospectively reviewed.
RESULTS: Forty-six Xp11.2 translocation RCC patients were identified from 4218 renal tumour patients who were underwentunderwent surgery in our centrecentre from Jan. 2014 to Apr. 2020. The incidence of Xp11.2 translocation RCCs in our centre was 1.09%. During a median follow-up period of 30.5 months, 4 patients died of the disease. The total median overall survival and cancer specific survival were 30.0 months and 24.0 months, respectively. The 1-year, 3-year and 5-year OS rates were 97.4%, 88.8%, and 88.8%, respectively. In multivariable analysis, displaying symptoms when diagnosed (p = 0.019), lymph node metastasis (p = 0.002) and distal metastasis (p = 0.020) were identified as risk factors for poor prognosis.
CONCLUSIONS: Xp11.2 translocation RCC is a type of renal cell carcinoma with a relatively low incidence and various prognoses. Early-stage Xp11.2 translocation RCCs have a similar prognosis to most typical RCCs, but late-stage Xp11.2 translocation RCCs can lead to poor oncological outcomes.