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Multidisciplinary management of pediatric malignant rhabdoid tumor based on 5 years of experience at a tertiary care center

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Abstract

Objective

Malignant rhabdoid tumor (MRT) is a highly aggressive tumor that occurs mostly in young children with extremely poor prognosis. Standardized and effective treatment strategies for MRT have yet to be established because of its rarity. Here, we report our single-institutional experience involving MRT treatment.

Methods

Patients with newly diagnosed MRT between March 2016 and October 2021 were included. The clinical characteristic, treatment-related toxicities, clinical outcomes, and prognostic factor were retrospectively analyzed.

Results

A total of 18 patients with MRT were enrolled during the 5 years. The median age was 42.8 months (range 10 to 82 years). Among the 18 patients, 9 patients died after a median of follow-up 26 months (range 3 to 42 months). The 1-year event-free survival (EFS) and 3-year overall survival (OS) rates of the entire cohort were 63% (95% CI 46% to 74%) and 67% (95% CI 49% to 82%), respectively. Univariate analysis of patients who underwent gross or total resection followed by adjuvant chemotherapy and radiotherapy demonstrated an improvement in 1-year EFS. However, only gross resection and total resection predicted a better 3-year OS.

Conclusions

Surgical excision is still the mainstream treatment for MRT. Postoperative adjuvant treatments including chemotherapy and radiotherapy contribute to improved disease control rate. Our single-institute experience may provide insights into the multimodal treatment of MRT.

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Data availability statement

The clinical data supporting the conclusions of this manuscript will be made available by the authors.

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Acknowledgements

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Funding

This work was supported by Innovative Research Program of Xiangyang No.1 People’s Hospital (Grant No. XYY2021Q02), Platform Special Fund for Scientific Research of Xiangyang No. 1 People’s Hospital (Grant No. XYY2022P05), and Key projects of Xiangyang Science and Technology Bureau (2021YL26).

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Authors and Affiliations

  1. Department of Oncology, Xiangyang No. 1 People’s Hospital, Hubei University of Medicine, Jiefang Road No. 15, Xiangyang, 441000, Hubei, China

    Yidi Han, Youhong Dong & Dongdong Zhang

  2. Department of Orthopedics, Xiangyang No. 1 People’s Hospital, Hubei University of Medicine, Jiefang Road No. 15, Xiangyang, 441000, Hubei, China

    Kai Lian

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  1. Yidi Han
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Contributions

All authors contributed to the study conception and design. Dongdong Zhang and Kai Lian provided the direction of study. Youhong Dong collected and analyzed the data, and wrote the manuscript; Yidi Han helped collect the data and revised the manuscript. All the authors read and approved the final manuscript.

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Correspondence to Kai Lian or Dongdong Zhang.

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This study was approved by the Ethics and Scientific Committee of Hubei University of Medicine and was performed according to the Good Clinical Practice Guidelines and the Helsinki Declaration. All the patients gave written informed consent before enrollment.

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Han, Y., Dong, Y., Lian, K. et al. Multidisciplinary management of pediatric malignant rhabdoid tumor based on 5 years of experience at a tertiary care center. Pediatr Surg Int 39, 51 (2023). https://doi.org/10.1007/s00383-022-05338-1

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