Renal cell carcinoma (RCC) in childhood is a unique disease entity, representing only 2.3-6.6% of all renal tumors in children. Most experience with pediatric RCC is limited to case reports or case series consisting of relatively small numbers of cases. The aim of this study is to present four additional cases of this unique disease entity. The records of four patients presenting to our institution with renal cell carcinoma between 1986 and 2006 were examined. Only patients younger than 10 years were included. The clinical data included age, sex, signs and symptoms, surgery performed and clinical outcome. Data recorded from the pathology reports included tumor size, histologic subtype, grade (WHO), and stage (TNM). There were three boys and one girl aged between 11 months and 10 years at presentation. The classic triad of flank pain, gross hematuria and palpable abdominal mass were not encountered. A histopathological diagnosis of RCC was confirmed in all four cases without any positive lymph node involvement. Due to the increased detection of tumors with the use of imaging techniques such as ultrasound and computerized tomography, an increased number of incidentally diagnosed RCCs are found. The primary choice of the treatment of any stage of RCC is surgical excision. However, the preoperative diagnosis of tumor in children is difficult and the effects of chemotherapy, including immunotherapy, are unclear.
