Renal cell carcinoma in children: a clinicopathologic study / 中华泌尿外科杂志

ABSTRACT

Objective To discuss the unique biological,histological and clinical features of pediatric renal cell carcinoma (RCC).Methods A retrospective review and biological analysis of all RCC cases presenting to our hospital from January 1973 to March 2012 was undertaken.Results Twenty-nine RCC pediatric patients (16 boys,13 girls) with mean age of 9.6 (range 2.5-16.0) years were identified.The presentations included hematuria in 17 (58.6％) cases with 3 who developed hematuria after trauma,abdominal mass with hematuria in 3 (10.3％),abdominal mass in 3,abdominal pain in 2,abdominal pain with hematuria in 1,and incidentally finding in 3.The diameter of tumor was from 2.5 cm to 25.0 cm,mean 6.8 cm.According to TNM stage grouping system,16 cases were stage Ⅰ,10 stage Ⅲ,and 3 stage Ⅳ.Xp1 1.2 translocation RCC was identified in 21 patients,clear cell RCC 6,papillary RCC 2.Of the 29 cases,3 patients with the tumor less than 7-cm had nephron-sparing surgery.A 15-cm tumor was incompletely removed in 1 patient and another patient with a 25 cm× 18 cm×15 cm tumor had gross residual.Nephrectomy was performed for the affected kidney in the remaining 24 patients.Twenty-one patients (Xp1 1.2 translocation RCC 13 patients,clear cell RCC 6,papillary RCC only 2) were followed up from 1.5 to 34.0 years,18 were living well (T1N0M0 in 11 cases,T1N1M0 in 2,T2N1M0 in 3,T3N1M0 in 1 and T4N1M1 in 1) and 3 died of recurrence.Conclusions Although RCC is rare in children,pediatric RCC behaves in a distinct fashion compared with adult forms of RCC.Hematuria is the main symptom in pediatric RCC.Xp11.2 translocation RCC is the predominant form,associated with an advanced stage at diagnosis.Nephrectomy is the common treatment for RCC and nephron sparing surgery could be a reasonable option for patients with tumor smaller than 7 cm.For localized RCC (T1-2 N0-1 M0),simple kidney removal surgery is sufficient for treatment without lymph node dissection and postoperative adjuvant treatment.