RCC is a rare disease in children and adolescents. This neoplasm has a different clinical presentation in children compared with adults but the same outcome. In our experience, patients with localized disease could be cured by nephrectomy alone. Prospective studies in a larger number of patients are needed to confirm radiation therapy and biologic response modifiers as effective adjunct therapy in RCC stage III. The alternative therapy seems warranted in patients with advanced disease.

Though our experience is limited to a small number of patients, our treatment strategy for patients with MC is appears clinically useful and demonstrates how cooperation within the TREP project has enabled enough data to be collected to propose treatment recommendations for pediatric patients with this very rare tumor.

In children with unilateral renal tumors nephron sparing surgery minimizes the decrease in renal function following renal ablative surgery, and may be of benefit in children with a progressive renal function decrease, probably due to a congenitally reduced nephron complement.

A subset of children with unilateral renal tumor presents before and/or after nephrectomy, and not after nephron sparing surgery, with stage II chronic kidney disease, probably due to a reduced renal reserve capacity. Whether patients with preoperative renal dysfunction may benefit from nephron sparing surgery should be studied in a cooperative clinical trial setting.

No abstract