PURPOSE
To characterize the epidemiology, histology, radiographic features and surgical approach of pediatric and adolescent renal cell carcinoma (pRCC).
PATIENTS AND METHODS
pRCC cases prospectively enrolled on the Children's Oncology Group study AREN03B2 underwent central pathology, radiology, surgery and oncology review.
RESULTS
As of June 2012, of 3250 patients enrolled on AREN03B2, 120 (3.7%) had unilateral RCC (median age 12.9 years, range 1.9-22.1; 52.5% female). Central review classified these as translocation morphology (56), papillary (20), renal medullary carcinoma (13), chromophobe (4), oncocytoma (1), conventional clear cell (1), and RCC not otherwise specified (25). Lymph node (LN) involvement (N+) was found in 35/73 (47.9%) cases for which LN were sampled, including 19/40 (47.5%) with primary tumors < 7cm. Using a size cut-off of 1cm, imaging detection of N+ had a sensitivity of 57.14% [(20/35); 95% CI: 39.35%, 73.68%] and specificity of 94.59% [(35/37); 95% CI: 81.81%, 99.34%]. Distant metastases were present in 23 (19.2%). Initial surgery was radical nephrectomy (RN) in 88 (73.3%), nephron-sparing surgery (NSS) in 18 (15.0%), and biopsy in 14 (11.7%). Patients undergoing NSS compared to RN were less likely to have LNs sampled (6/18 (33.3%) vs. 65/88 (73.9%), p-value = 0.002).
CONCLUSIONS
Translocation RCC is the most common form of pediatric and adolescent RCC. Nodal disease is common and seen with small primary tumors. Imaging has a high specificity but relatively low sensitivity for detection of such lymph node disease. Failure to sample LNs results in incomplete staging and potentially inadequate disease control for younger RCC patients.