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Establishment and characterization of a recurrent malignant peripheral nerve sheath tumor cell line: RsNF

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Abstract

Malignant peripheral nerve sheath tumor (MPNST) is a highly aggressive and recurrent soft tissue sarcoma. It most commonly occurs secondary to neurofibromatosis type I, and it has a 5-year survival rate of only 8–13%. To better study the tumor heterogeneity of MPNST and to develop diverse treatment options, more tumor-derived cell lines are needed to obtain richer biological information. Here, we established a primary cell line of relapsed MPNST RsNF cells derived from a patient diagnosed with NF1 and detected the presence of NF1 mutations and SUZ12 somatic mutations through whole-exome sequencing(WES). Through tumor molecular marker targeted sequencing and single-cell transcriptome sequencing, it was found that chromosome 7 copy number variation (CNV) was gained in this cell line, and ZNF804B, EGFR, etc., were overexpressed on chromosome 7. Therefore, RsNF cells can be used as a useful tool in NF1-associated MPNST genomic amplification studies and to develop new therapeutic strategies.

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Acknowledgements

We thank Beijing Tiantan Hospital for providing clinical specimens and pathologists for histopathological classification and immunohistochemical guidance in this article.

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Platform Construction of Basic Research and Clinical Translation of Nervous System Injury (PXM2021_026280_000006, Beijing Municipal Health Commission).

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Authors and Affiliations

  1. Beijing Key Laboratory of Central Nervous System Injury, Beijing Neurosurgical Institute, Capital Medical University, Beijing, China

    Xingnan Zhang, Chenhao Hu & Song Liu

  2. Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, China

    Dezhi Li

  3. U1195, Inserm et Universite Paris-Saclay, 94276, Le Kremlin-Bicetre, France

    Song Liu

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XZ contributed to the conception and design of the study and the experimental operations. CH contributed to the acquisition and analysis of data. DL and SL contributed to the drafting of the text and the preparation of the figures. All authors contributed to the article and approved the submitted version.

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Correspondence to Song Liu.

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This study was approved by the ethical committee of the National Cancer Center, and all patients in this study provided written informed consent (2004050).The study using animal models was approved by the ethics committee of Beijing Tiantan Hospital Ethics Committee of Capital Medical University (BNI202304002).

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Zhang, X., Hu, C., Li, D. et al. Establishment and characterization of a recurrent malignant peripheral nerve sheath tumor cell line: RsNF. Human Cell 37, 345–355 (2024). https://doi.org/10.1007/s13577-023-01000-7

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