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Published in: BMC Pediatrics 1/2022

Open Access 01-12-2022 | Metastasis | Research

Clinical and prognostic analysis of 42 children with malignant rhabdoid tumor of the kidney: a 7-year retrospective multi-center study

Authors: Chenghao Zhanghuang, Zhaoxia Zhang, Li Zeng, Bing Yan, Haoyu Tang, Jinkui Wang, Xing Liu, Guanghui Wei, Dawei He

Published in: BMC Pediatrics | Issue 1/2022

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Abstract

Objective

To discuss the clinical and prognostic indicators of pediatric malignant rhabdoid tumor of the kidney (MRTK), and to increase the understanding of the occurrence and development of MRTK.

Methods

From July 2014 to September 2021, all cases were confirmed by postoperative pathological examination. Among the 42 patients, there were 25 males and 17 females, with a median age of 10 (1–84) months. Abdominal mass or hematuria were the main clinical manifestations. Preoperative chemotherapy was performed in 9 cases (VC). The tumor stages were stage I-IV. Preoperative metastasis was found in 9 cases; the most common site was the lung. Postoperative patients received conventional chemotherapy, including VDACE regimen and UH-1 regimen. Among the 42 children in this group, survival at follow-up in this study was 26.2%(11/42).

Results

Preoperative anemia was found by univariate analysis, hypertension and hypercalcemia had shorter survival time. In addition, tumor-related factors had a significant impact on survival, with incomplete tumor resection, lymph node metastasis, stage III-IV had a lower survival rate. The impact of postoperative factors on survival included postoperative complications had a lower survival rate. The children were younger than 12 months, preoperative metastasis, no chemotherapy was performed after surgery was an independent risk factor for the prognosis of MRTK.

Conclusion

The main clinical manifestations about MRTK were abdominal mass and hematuria. Preoperative chemotherapy did not significantly improve the prognosis. Postoperative chemotherapy can significantly improve the survival rate. Diagnosis depends on clinical manifestations, imaging, histopathology, immunohistochemistry and other comprehensive judgment. Age less than 12 months, preoperative metastasis, and no postoperative chemotherapy were independent risk factors for prognosis.
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Metadata
Title
Clinical and prognostic analysis of 42 children with malignant rhabdoid tumor of the kidney: a 7-year retrospective multi-center study
Authors
Chenghao Zhanghuang
Zhaoxia Zhang
Li Zeng
Bing Yan
Haoyu Tang
Jinkui Wang
Xing Liu
Guanghui Wei
Dawei He
Publication date
01-12-2022
Publisher
BioMed Central
Published in
BMC Pediatrics / Issue 1/2022
Electronic ISSN: 1471-2431
DOI
https://doi.org/10.1186/s12887-022-03643-1

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