Kyung-Nam Koh, Jung Woo Han, Hyoung Soo Choi, Hyoung Jin Kang, Ji Won Lee, Keon Hee Yoo, Ki Woong Sung, Hong Hoe Koo, Kyung Taek Hong, Jung Yoon Choi, Sung Han Kang, Hyery Kim, Ho Joon Im, Seung Min Hahn, Chuhl Joo Lyu, Hee-Jo Baek, Hoon Kook, Kyung Mi Park, Eu Jeen Yang, Young Tak Lim, Seongkoo Kim, Jae Wook Lee, Nack-Gyun Chung, Bin Cho, Meerim Park, Hyeon Jin Park, Byung-Kiu Park, Jun Ah Lee, Jun Eun Park, Soon Ki Kim, Ji Yoon Kim, Hyo Sun Kim, Youngeun Ma, Kyung Duk Park, Sang Kyu Park, Eun Sil Park, Ye Jee Shim, Eun Sun Yoo, Kyung Ha Ryu, Jae Won Yoo, Yeon Jung Lim, Hoi Soo Yoon, Mee Jeong Lee, Jae Min Lee, In-Sang Jeon, Hye Lim Jung, Hee Won Chueh, Seunghyun Won, the Korean Pediatric Hematology and Oncology Group (KPHOG)
Cancer Res Treat. 2023;55(1):279-290. Published online August 11, 2022
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.
Citations
Citations to this article as recorded by
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Loss of YTHDF1 suppresses the progression of malignant rhabdoid tumor of the kidney by regulating Glutathione S-transferase Mu 2 (GSTM2) Qian-Wen Xiong, Yuntao Liu, Min He, Xiao-Die Shen, Manli Zhao, Shuang-Ai Liu, Gensheng Zhang, Qian Liu, Jinhu Wang, Wan-Xin Peng Cell Biology and Toxicology.2025;[Epub] CrossRef
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Hyoung Soo Choi, Hyoung Jin Kang, Jun Ah Lee, Hyo Heong Han, Hyeon Jin Park, Eun Sun Yoo, Woo Sun Kim, Hee Young Shin, In One Kim, Sang Hoon Lee, Hyo Seop Ahn, Han Koo Lee
PURPOSE This study was performed to determine the outcome after treatment of osteosarcoma with intraarterial cisplatin as a preoperative chematherapy regimen. MATERIALS AND METHODS Twenty five patients with extremity osteosarcoma were treated with intraarterial cisplatin at Seoul National University Children's Hospital from January 1987 to April 1996. The dose of cisplatin was 130 mg/m2 and three to six courses were repeated two- to three-week intervals, Systemic doxorubicin was added to six of these patients. This was followed by surgical resection(limb salvage or amputation) and postoperative adjuvant chemotherapy. RESULTS Limb-salvage was possible in twenty of these twenty five patients. Pulmonary metastasis was present in five patients at diagnosis and developed later in three patients.
In six patients treated with systemic doxorubicin, pulmonary metastasis was absent at diagnosis and during follow-up period. Local recurrence after limb salvage was occurred in one patient and treated with amputation and systemic chemotherapy. Seven patients died from pulmonary metastssis and one from unknown cause. The follow-up duration of these patients was three to eighty eight months(median twenty two months) and the overall five-year survival and event free survival rate were 62.1% and 57.5%, respectively. CONCLUSION These data demonstrate that intraarterial cisplatin can be used as an effective regimen preoperatively for pediatric patients with extremity osteosarcoma. The combined use of systemic doxorubicin is expected to improve survival in patients with pulmonary metastasis.