ABSTRACT
Objective: To analyze the clinical characteristics, treatment methods and prognosis of renal cell carcinoma associated with Xp11.2 translocation/TFE3 gene fusions (Xp11.2 tRCC). Methods: From January 2007 to February 2018, 48 patients were diagnosed with Xp11.2 tRCC at Nanjing Drum Tower Hospital. The epidemiological features, treatment methods and long-term follow-up results were retrospectively reviewed. Results: Of the 48 patients, 20 cases were female and 28 cases were male, aged from 2 to 72 years. Gross hematuria and flank pain were the most frequent symptoms, which occurred on 14 cases and 8 cases respectively. The mean tumor size of 48 cases was (5.3±2.5)cm. Among the 34 cases who were classified as stageâ /â ¡, 14 cases received laparoscopic nephron-sparing surgery(NSS)and 20 cases received radical nephrectomy(RN). The other 14 cases who were classified as stage â ¢/â £ received RN but one case received target therapy. On univariate analysis, tumor diameter, adjuvant treatment, AJCC stage, lymph node metastasis and vein tumor thrombosis showed association with progression-free survival (PFS) and overall survival (OS) (P<0.05). Multivariate analysis indicated that AJCC stage (P=0.023, 95% CI: 0.048-0.081)and vein tumor thrombosis (P=0.046, 95% CI: 1.004-1.590)were independent prognostic factors of PFS. Conclusions: Xp11.2 tRCC mainly occurs in females. RN was the major method for Xp11.2 tRCC. However, NSS can also receive satisficed results for stage T1a case. High AJCC stage and the occurrence of vein tumor thrombosis indicated poor prognosis.
Subject(s)
Basic Helix-Loop-Helix Leucine Zipper Transcription Factors/genetics , Carcinoma, Renal Cell , Kidney Neoplasms , Adolescent , Adult , Aged , Carcinoma, Renal Cell/genetics , Child , Child, Preschool , Chromosomes, Human, X , Female , Gene Fusion , Humans , Kidney Neoplasms/genetics , Male , Middle Aged , Retrospective Studies , Translocation, Genetic , Young AdultABSTRACT
Objective: To explore the safety and clinical efficacy of partial nephrectomy for solitary kidney tumor. Methods: Twenty patients with a functional or anatomic solitary kidney who underwent nephron-sparing surgery for one or more renal masses were retrospectively analyzed. There were 15 male and 5 female patients with mean age of 63 years.Patients were divided into open partial nephrectomy(OPN) group and laparoscopic partial nephrectomy(LPN) group, the two groups were followed up.Demographic and clinical characteristics were analyzed using student t-test for continuous variables, and the χ2 test for categorical variables. Results: There were 21 tumors whose maximum diameter were 1.5 to 6.0 cm, the mean size was (3.7±1.3)cm, mean operative time was (205.3±81.8)minutes, and median estimated blood loss was 385.6 ml.Five cases accepted blood transfusion intro-operation, 1 case appeared leakage of urine post-operation, none had obvious fever.Eighteen cases were clear cell carcinoma, 2 cases were chromophobe renal carcinoma, 1 case was papillary renal cell carcinoma.Seventeen cases were T1aNxM0, 3 cases were T1bNxM0, 1 case was T3NxM0.One case lost to follow up, the others had completed a mean follow-up of 21.5 months (ranging from 1 to 53 months). One case recurred, no one had metastasis, all patients were alive.The hemoglobin ((114.9±19.6)g/L) and estimated glomerular filtration rate(eGFR) ((46.5±18.2) ml/min) of postoperative day were lesser than pre-operation ((130.7±18.8)g/L, (58.0±16.4) ml/min), while the serum creatinine was higher ((123.8±39.8) µmol/L vs.(90.9±33.2) µmol/L). Three months after operation, hemoglobin, serum creatinine and eGFR had no difference with pre-operative.Compared with OPN group, LPN group gain less blood loss (306 ml vs.510 ml)(t=-2.38, P=0.03), had shorter length of stays (15 d vs.21 d)(t=-3.34, P=0.04), had longer time of renal artery clamping (25.8 min vs.16.5 min)(t=2.60, P=0.02). Conclusions: Partial nephrectomy is secure and effective selection for solitary kidney tumor.The LPN has less trauma, gain less blood loss, recover faster and has shorter length of stays than the OPN, which needs shorter ischaemic time.