Presentation Authors: Ning Liu*, Nanjing, China, People's Republic of, Weidong Gan, Nanjing, , China, People's Republic of
Introduction: Renal cell carcinoma with Xp11.2 translocation (Xp11.2 tRCC) was a type of rare tumor. To investigate the clinicopathologic characteristics, treatments and prognosis of Xp11.2 tRCC.
Methods: All patients which were collected from five institutions were diagnosed by immunohistochemistry staining of TFE3 and fluorescence in situ hybridization between January 2007 and September 2018. Their clinicopathologic features, treatment methods and prognosis were retrospectively reviewed. Kaplanâ€“Meier methods were performed to estimate survival, and Cox proportional hazards model was used to evaluate the predictive role of the factors.
Results: In total, 72 cases of Xp11.2 tRCC were identified. Of the 72 enrolled patients, 31(43.1%) were female and 41(56.9%) were male, who aged from 2 to 72 years. Gross hematuria and abdominal mass were frequent symptoms. The mean tumor size was 5.7Â±2.6 centimeters. Among the 48(66.7%) patients who were classified as stage â… /â…¡, 19(39.6%) cases received nephron-sparing surgeryï¼ˆNSSï¼‰and 29(60.4%) cases received radical nephrectomy (RN). The other 24(33.3%) patients who were classified as stage â…¢/ â…£ received RN. Vena caval tumor thrombectomy was simultaneously performed on 8 (11.1%) patients who had vena cava tumor thrombosis. After operation, 31 of 48 (64.6%) patients in stage â… /â…¡ received adjuvant immunotherapy (Interleukin-2/Interferon-Î±), and 23 of 24 (95.8%) patients in stage â…¢/ â…£ received adjuvant VEGF-targeted therapy (Sorafenib/ Sunitinib). Three-year overall survival rate (OS) and progression-free survival rate (PFS) of patients in stage â…¢/ â…£ were 51.1% and 57.4%. which contrast to patients in stage â… /â…¡ were 97.2% and 87.4%, respectively. There was no significance between NSS and RN for patients in stage T1a.On univariate analysis, tumor size, adjuvant treatment, pathological stage and vena cava tumor thrombosis showed association with both PFS and OS but lymph node metastasis is only related to OS (all P < 0.05). Multivariate analysis indicated that pathological stage (P=0.009) and vena cava tumor thrombosis (P=0.006) were independent prognostic factors of PFS, and that vena cava tumor thrombosis (P=0.015) and lymph node metastasis (P=0.002) showed independent prognostic significance of OS.
Conclusions: Xp11.2 tRCC mainly occurs in young females. NSS can be performed for cases in stage T1a, otherwise, RN should be considered. The occurrence of vein tumor thrombosis and advance stage indicated poor prognosis.
Source of Funding: National Natural Science Foundation of China (81572512)