The Clinicopathological Characteristics and Prognosis of 55 Patients With TFE3-Rearranged Renal Cell Carcinomas

IF 2.3 3区医学 Q3 ONCOLOGY

Clinical genitourinary cancer Pub Date : 2024-07-16 DOI:10.1016/j.clgc.2024.102165

Yin-Miao Bai , Li Yang , Yue Yang , Xiang-Xu Wang , Meng-Di Zheng , Xiao Chai , Qiong-Yi Dou , Hong-Mei Zhang

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引用次数: 0

Abstract

Objective

To explore the clinicopathological features and prognosis of TFE3-rearranged renal cell carcinomas (TFE3-rRCC).

Methods

In this retrospective observational study, the data of patients with TFE3-rRCC admitted to Xijing Hospital from January 2010 to October 2023 were collected, encompassing the general information, pathological diagnosis, immunohistochemistry, and the results of FISH detection. The treatment information and survival data of the patients were recorded during the follow-up.

Results

A total of 55 patients with TFE3-rRCC were enrolled, among whom 25 were males and 30 were females. TFE3 FISH assay suggested the disruption of the TFE3 gene. Fifty-four patients underwent surgical resection of kidney lesions, while 1 patient did not. By the end of follow-up in December 2023, 3 patients were lost to follow-up, 28 patients remained alive, and 24 patients had died. Among the 52 patients followed up, 31 developed metastases, involving lymph nodes, liver, bone, lung, peritoneum, pleura, adrenal gland, and brain. The 1-year and 5-year survival rates of the patients were 84.6% and 50.6%, respectively. In this study, there were 31 patients with TFE3-rRCC recurrence or metastasis. Median PFS was 7 and 13 months in the VEGFR-TKI and VEGFR-TKI+ ICI groups, respectively. The median OS was 12 months in the VEGFR-TKI treatment group. The median OS data of VEGFR-TKI+ ICI group has not been reached. The ORR and DCR was 25%, 66.7% in the VEGFR-TKI group. The ORR and DCR was 33.3%, 77.8% in the VEGFR-TKI+ ICI group.

Conclusion

TFE3-rRCC is a rare subtype of malignant renal tumor. The diagnosis mainly relies on pathological morphology, immunohistochemistry, and the detection of TFE3 gene disruption by FISH. In terms of treatment, surgery is the primary approach, and lymph nodes, liver, and bone are the main metastatic sites. VEGFR-TKI+ICI treatment might be an option of recurrent or metastatic TFE3-rRCC.

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55例TFE3重排肾细胞癌患者的临床病理特征和预后

方法收集2010年1月至2023年10月西京医院收治的TFE3-rRCC患者资料，包括一般资料、病理诊断、免疫组化及FISH检测结果。结果共纳入 55 例 TFE3-rRCC 患者，其中男性 25 例，女性 30 例。TFE3 FISH检测表明TFE3基因被破坏。54名患者接受了肾脏病变手术切除，1名患者未接受手术切除。到 2023 年 12 月随访结束时，3 名患者失去了随访机会，28 名患者仍然存活，24 名患者死亡。在随访的 52 名患者中，31 人出现转移，涉及淋巴结、肝、骨、肺、腹膜、胸膜、肾上腺和脑。患者的 1 年和 5 年生存率分别为 84.6% 和 50.6%。在这项研究中，TFE3-rRCC复发或转移的患者有31例。VEGFR-TKI组和VEGFR-TKI+ ICI组的中位PFS分别为7个月和13个月。VEGFR-TKI治疗组的中位OS为12个月。VEGFR-TKI+ICI组的中位OS数据尚未达到。VEGFR-TKI组的ORR和DCR分别为25%和66.7%。VEGFR-TKI+ICI组的ORR和DCR分别为33.3%和77.8%。结论TFE3-rRCC是一种罕见的肾脏恶性肿瘤亚型，诊断主要依靠病理形态学、免疫组化和FISH检测TFE3基因干扰。在治疗方面，手术是主要方法，淋巴结、肝脏和骨骼是主要转移部位。VEGFR-TKI+ICI治疗可能是复发或转移性TFE3-rRCC的一种选择。

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来源期刊

Clinical genitourinary cancer 医学-泌尿学与肾脏学

CiteScore

5.20

自引率

6.20%

发文量

201

审稿时长

54 days

期刊介绍： Clinical Genitourinary Cancer is a peer-reviewed journal that publishes original articles describing various aspects of clinical and translational research in genitourinary cancers. Clinical Genitourinary Cancer is devoted to articles on detection, diagnosis, prevention, and treatment of genitourinary cancers. The main emphasis is on recent scientific developments in all areas related to genitourinary malignancies. Specific areas of interest include clinical research and mechanistic approaches; drug sensitivity and resistance; gene and antisense therapy; pathology, markers, and prognostic indicators; chemoprevention strategies; multimodality therapy; and integration of various approaches.