EPIDEMIOLOGIC AND CLINICAL OUTCOMES OF PEDIATRIC RENAL TUMORS IN KOREA: A RETROSPECTIVE ANALYSIS OF THE KOREAN PEDIATRIC HEMATOLOGY AND ONCOLOGY GROUP (KPHOG) DATA

초록

Background and Aims: Renal tumors account for approximately 7% of all childhood cancers, including Wilms tumor(WT), clear cell sarcoma of the kidney(CCSK), malignant rhabdoid tumor(MRTK), renal cell carcinoma(RCC), congenital mesoblastic nephroma(CMN) and other rare tumors. We investigated the epidemiology of pediatric renal tumors in Korea. Methods: From January 2001 to December 2015, data of pediatric patients (0–18 years of age) newly-diagnosed with renal tumors at 26 hospitals were retrospectively analyzed. Results: Among 439 patients (male 240), the most common tumor was WT, at 77.9% (n=342), followed by RCC, CCSK, MRTK, CMN,and others at 8.2% (n=36), 5.5% (n=24), 3.6% (n=16), 2.7% (n=12), and 2.1% (n=9), respectively. Median age at diagnosis was 27.1 months (range 0-225.5) and median follow-up duration 88.5 months (range 0-211.6). Overall, 32 patients died, of whom 17, 11, 1, and 3 died of relapse, progressive disease, second malignant neoplasm, and treatment-related mortality. Five-year overall survival and event free survival were 97.2% and 84.8% in WT, 90.6% and 82.1% in RCC, 81.1% and 63.6% in CCSK, 60.3% and 56.2% in MRTK, and 100% and 91.7% in CMN, respectively (P < 0.001). Conclusions: The pediatric renal tumor types in Korea are similar to those of previous reports in other countries. WT accounted for a large proportion and survival was excellent. Non-Wilms renal tumor included a variety of tumors and showed inferior outcome especially in MRTK. Further efforts are necessary to optimize the treatment and to analyze the genetic characteristics of pediatric renal tumors in Korea.