Cancer Management and Research (Jun 2021)
Case Analysis of 14 Children with Malignant Rhabdoid Tumor of the Kidney
Abstract
Jing Li,* Weiling Zhang,* Huimin Hu, Yi Zhang, Yizhuo Wang, Huali Gu, Dongsheng Huang Department of Pediatrics, Beijing Tongren Hospital of China Capital Medical University, Beijing, 100176, People’s Republic of China*These authors contributed equally to this workCorrespondence: Dongsheng HuangDepartment of Pediatrics, Beijing Tongren Hospital of China Capital Medical University, No. 1 Dongjiao Lane, Dongcheng District, Beijing, 100730, People’s Republic of ChinaTel +86 10 5826 6032Email [email protected]: This study aims to summarize the clinical features and prognoses of the malignant rhabdoid tumor of the kidney (MRTK) in children. It further aims to analyze the high-risk factors affecting MRTK prognosis.Methods: Clinical data from 14 children with MRTK treated in Paediatrics of Beijing Tongren Hospital from January 2010 to December 2019, along with the high-risk factors affecting prognosis, were retrospectively analyzed.Results: There were 14 children with MRTK included in the study, with a median onset age of 13 (3– 46) months. Thirteen patients had distant metastases, the most common site for metastases being inside the lung. A comprehensive treatment protocol combined with chemotherapy was mainly applied during the surgery. A surgical resection of primary tumors was performed on 13 (13/14) patients, and all 14 children received chemotherapy with ifosfamide + carboplatin + etoposide, ifosfamide + etoposide, and vincristine + pirarubicin + cyclophosphamide regimens, alternately. Three patients received radiotherapy and two received oral targeted drugs after partial response. The median follow-up was after 16.5 months (3– 53 months) and the four-year overall survival (OS) was 41.8%. In children aged ≤ 24 months and children aged > 24 months, the two-year OS was 67.2% and 100% (χ2 = 108.998, P< 0.05), respectively. In children with Ki 67 > 70% and children with Ki 67 < 70%, the two-year OS was 52.6% and 86.9% (χ2 = 8.544, P = 0.003), respectively. In children with distant metastases and children without distant metastasis, the two-year OS was 70% and 100% (χ2 = 14.239, P< 0.05), respectively.Conclusion: The most common MRTK distant metastasis site is the lung. Risk factors for poor MRTK prognoses include an age of < 24 months, Ki 67 > 70%, and distant metastases.Keywords: children, malignant rhabdoid tumor of the kidney, MRTK, treatment, prognosis
