While the incidence of renal cell carcinoma (RCC) is rising, it remains a relatively uncommon malignancy in the pediatric population, where it represents only 2-6% of solid renal malignancies.
In this report, Cook and colleagues describe their experience with the management of RCC in childhood, specifically examining the role of partial nephrectomy as a reasonable surgical approach in patients with anatomically amenable tumors.
Fifteen patients with RCC were seen over a 25 year period. Mean patient age was 7.9 years (range 2.5-18 years). Seventy-five percent of patients presented with symptoms related to their local tumor (gross hematuria, abdominal pain, and polycythemia) whereas 25% had their tumors detected based on radiographic imaging for other reasons or physical examination. In this series, 10 patients were treated with radical nephrectomy, whereas 5 patients were treated with partial nephrectomy. Six patients had localized disease (Robson Stage I), 8 patients had locally advanced disease (Robson II and III), and one patient had metastatic disease (Robson Stage IV) at presentation. With regard to histology, 8 patients had papillary RCC, whereas 7 had conventional clear cell. Seventy-three percent of patients had low grade disease (Fuhrman grade I or II). Three patients either presented with or developed metastatic disease. Overall, 14 patients (93.3%) were alive at a mean follow-up of 4.9 years. All but one patient remains recurrence free, including all patients who underwent nephron sparing surgery.
In the pediatric population, RCC remains a rare malignancy that appears to have an overall favorable prognosis, even in more advanced disease. When anatomically feasible, nephron sparing surgery in the management of the primary tumor in pediatric patients seems to be a reasonable treatment approach that provides adequate local tumor control.
By Christopher G. Wood, MD
Reference:
J Urol 175: 1456-1460, 2006
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