Clinical characteristics of XP11.2 translocation/TFE3 gene fusion renal cell carcinoma: a systematic review and meta-analysis of observational studies.

Xiangming Cheng,Gutian Zhang,Xiaogong Li,Weidong Gan,Hongqian Guo

doi:10.1186/s12894-016-0154-6

Xiangming Cheng, Gutian Zhang + Show 3 more

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https://doi.org/10.1186/s12894-016-0154-6

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Journal: BMC urology	Publication Date: Jul 11, 2016
Citations: 58	License type: cc-by

Affiliation: Nanjing Drum Tower Hospital

Abstract

BackgroundRenal cell carcinoma (RCC) associated with Xp11.2 translocation/TFE3 gene fusion (Xp11.2 RCC) is a rare subtype of RCC which is firstly described as a distinct entity in 2004 so that clinical characteristics of Xp11.2 RCC in different gender and age are unknown. The purpose of systematic review and meta-analysis is to provide a comprehensive assessment on them.MethodsMEDLINE, EMBASE and Cochrane databases were searched for studies which evaluate the clinical characteristics of Xp11.2 RCC. The literature published between July 2004 and May 2014 was searched.ResultsA total of 15 studies with 147 participants were included. The meta-analysis demonstrated that number of patients of all age in female was higher than in male with pooled OR of 3.93(95 % CI = 1.66–9.34). However, incidence of distant metastases (OR = 0.34, 95 % CI = 0.12–1.57) and lymphatic metastases (OR = 0.51, 95 % CI = 0.14–1.91), tumor stage (OR = 0.85, 95 % CI = 0.34–2.15) and overall survival (OS) (OR = 0.46, 95 % CI = 0.05–4.34) between male and female were comparable. Incidence in female was higher than in male with pooled OR of 5.13(95 % CI = 1.67–15.72) in adults, while in children no gender-related predominance (OR = 1.19, 95 % CI = 0.38–3.72) was observed. In addition, incidence of distant metastases (OR = 1.00, 95 % CI = 0.13–7.84) and lymphatic metastases (OR = 1.00, 95 % CI = 0.07–13.67) and tumor stage (OR = 1.94, 95 % CI = 0.20–19.03) between children and adults were comparable. Survival curves presented comparable outcomes between male and female (P = 0.707) as well as between children and adults (P = 0.383).ConclusionsFemale patients with Xp11.2 RCC in adults exhibit a high incidence compared to male, but not in children. Comparable clinical characteristics including incidence of distant and lymphatic metastases, tumor stage and prognosis is presented between male and female as well as between children and adults.

Highlights

Renal cell carcinoma (RCC) associated with Xp11.2 translocation/transcription factor E3 (TFE3) gene fusion (Xp11.2 RCC) is a rare subtype of RCC which is firstly described as a distinct entity in 2004 so that clinical characteristics of Xp11.2 RCC in different gender and age are unknown
Renal cell carcinoma (RCC) associated with Xp11.2 translocation/TFE3 gene fusion (Xp11.2 RCC) is a rare subtype of RCC which is delineated as a distinct entity in the 2004 World Health Organization renal tumor classification [1]
As Xp11.2 RCC predominantly among children and associated with a poorer prognosis, the comparison of the prognosis between children and adults exists as well [9]. Due to such controversy in gender and age of Xp11.2 RCC, in this systematic review and meta-analysis, we studied the clinical characteristics of Xp11.2 RCC regarding patient demographics, incidence of distant and lymphatic metastases, tumor stage and prognosis in order to better define the difference of Xp11.2 RCC between male and female as well as between children and adults

Summary

Introduction

Renal cell carcinoma (RCC) associated with Xp11.2 translocation/TFE3 gene fusion (Xp11.2 RCC) is a rare subtype of RCC which is firstly described as a distinct entity in 2004 so that clinical characteristics of Xp11.2 RCC in different gender and age are unknown. Renal cell carcinoma (RCC) associated with Xp11.2 translocation/TFE3 gene fusion (Xp11.2 RCC) is a rare subtype of RCC which is delineated as a distinct entity in the 2004 World Health Organization renal tumor classification [1]. This subtype affects primarily children more than adults, accounts for 20–40 % of pediatric RCC and 1–1.6 % of RCC in adults [2]. Xp11.2 RCCs are generally characterized by several translocations on chromosome Xp11.2 resulting in a gene fusion between TFE3 and at least 6 possible partners [4] Since these translocations are located on the X chromosome, it seems reasonable to suggest that there are gender differences in the clinical characteristics of Xp11.2 RCC. Comparative analysis regarding the clinical course of Xp11.2 RCC between male and

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Conclusion