Cancer Res Treat.  2023 Jan;55(1):279-290. 10.4143/crt.2022.073.

Epidemiologic and Clinical Outcomes of Pediatric Renal Tumors in Korea: A Retrospective Analysis of The Korean Pediatric Hematology and Oncology Group (KPHOG) Data

• Koh KN
• Han JW
• Choi HS
• Kang HJ
• Lee JW
• Yoo KH
• Sung KW
• Koo HH
• Hong KT
• Choi JY
• Kang SH
• Kim H
• Im HJ
• Hahn SM
• Lyu CJ
• Baek HJ
• Kook H
• Park KM
• Yang EJ
• Lim YT
• Kim S
• Lee JW
• Chung NG
• Cho B
• Park M
• Park HJ
• Park BK
• Lee JA
• Park JE
• Kim SK
• Kim JY
• Kim HS
• Ma Y
• Park KD
• Park SK
• Park ES
• Shim YJ
• Yoo ES
• Ryu KH
• Yoo JW
• Lim YJ
• Yoon HS
• Lee MJ
• Lee JM
• Jeon IS
• Jung HL
• Chueh HW
• Won S
• the Korean Pediatric Hematology and Oncology Group (KPHOG)

• KMID: 2538011
• DOI: http://doi.org/10.4143/crt.2022.073

Abstract

Purpose
Renal tumors account for approximately 7% of all childhood cancers. These include Wilms tumor (WT), clear cell sarcoma of the kidney (CCSK), malignant rhabdoid tumor of the kidney (MRTK), renal cell carcinoma (RCC), congenital mesoblastic nephroma (CMN) and other rare tumors. We investigated the epidemiology of pediatric renal tumors in Korea.
Materials and Methods
From January 2001 to December 2015, data of pediatric patients (0–18 years) newly-diagnosed with renal tumors at 26 hospitals were retrospectively analyzed.
Results
Among 439 patients (male, 240), the most common tumor was WT (n=342, 77.9%), followed by RCC (n=36, 8.2%), CCSK (n=24, 5.5%), MRTK (n=16, 3.6%), CMN (n=12, 2.7%), and others (n=9, 2.1%). Median age at diagnosis was 27.1 months (range 0-225.5) and median follow-up duration was 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).
Conclusion
The pediatric renal tumor types in Korea are similar to those previously reported in other countries. WT accounted for a large proportion and survival was excellent. Non-Wilms renal tumors included a variety of tumors and showed inferior outcome, especially MRTK. Further efforts are necessary to optimize the treatment and analyze the genetic characteristics of pediatric renal tumors in Korea.

Keyword

Kidney neoplasms; Child; Epidemiology

Figure

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  Fig. 1 Pediatric renal tumors in Korea. (A) Proportion of tumor types. (B) Age distribution of tumor types. CCSK, clear cell sarcoma of the kidney; CMN, congenital mesoblastic nephroma; MRTK, malignant rhabdoid tumor of the kidney; RCC, renal cell carcinoma; WT, Wilms tumor.

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  Fig. 2 Kaplan-Meier survival curve for pediatric renal tumors. (A) Overall survival (OS) and event-free survival (EFS) for all tumor types. (B) OS by tumor types. (C) EFS by tumor types. CCSK, clear cell sarcoma of the kidney; CMN, congenital mesoblastic nephroma; MRTK, malignant rhabdoid tumor of the kidney; RCC, renal cell carcinoma; WT, Wilms tumor.

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  Fig. 3 Kaplan-Meier survival curve for Wilms tumor. (A) Overall survival of Wilms tumor by staging. (B) Event-free survival of Wilms tumor by staging.

Reference

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