Characterization of a pleomorphic rhabdomyosarcoma cell line
Abstract Pleomorphic rhabdomyosarcoma (PRMS) is an extremely rare soft tissue tumor with dismal prognosis that has a higher incidence in adults compared to the other RMS subtypes. The unique PRMS cell line BH1522 was established from a pleural effusion of a lung metastasis and the characteristics of...
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Nature Portfolio
2025-01-01
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| Series: | Scientific Reports |
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| Online Access: | https://doi.org/10.1038/s41598-025-87027-2 |
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| author | Sandra Stickler Clemens Lang Maximilian Rieche Marie-Therese Eggerstorfer Martin Hohenegger Maximilian Hochmair Gerhard Hamilton |
| author_facet | Sandra Stickler Clemens Lang Maximilian Rieche Marie-Therese Eggerstorfer Martin Hohenegger Maximilian Hochmair Gerhard Hamilton |
| author_sort | Sandra Stickler |
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| description | Abstract Pleomorphic rhabdomyosarcoma (PRMS) is an extremely rare soft tissue tumor with dismal prognosis that has a higher incidence in adults compared to the other RMS subtypes. The unique PRMS cell line BH1522 was established from a pleural effusion of a lung metastasis and the characteristics of this cell line were compared to two embryonal type RMS (ERMS) cell lines. The affected patient had been treated by surgery, several cycles of chemotherapy and thoracoscopy of the lung metastases. Chemosensitivity of the PRMS cell lines was checked using cytotoxicity assays and oncogene-related and phosphoproteins by Western blot arrays. The BH1522 cell line proved to be chemosensitive to conventional chemotherapeutics and the Son of Sevenless 1 homolog (SOS1) inhibitor BAY-293 that inhibited proliferation and suppressed MYC activity. Results of the novel functional BH1522 tests corroborate published tumor sequencing data that demonstrated alterations in receptor tyrosine kinase/MAPK, PI3K/AKT and mutated p53 as suspected drivers of malignant growth. |
| format | Article |
| id | doaj-art-9511e8d2f90c4703b75c8863ff205945 |
| institution | Kabale University |
| issn | 2045-2322 |
| language | English |
| publishDate | 2025-01-01 |
| publisher | Nature Portfolio |
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| series | Scientific Reports |
| spelling | doaj-art-9511e8d2f90c4703b75c8863ff2059452025-01-26T12:30:39ZengNature PortfolioScientific Reports2045-23222025-01-0115111210.1038/s41598-025-87027-2Characterization of a pleomorphic rhabdomyosarcoma cell lineSandra Stickler0Clemens Lang1Maximilian Rieche2Marie-Therese Eggerstorfer3Martin Hohenegger4Maximilian Hochmair5Gerhard Hamilton6Institute of Pharmacology, Medical University of ViennaDepartment of Trauma Surgery, Hospital DonaustadtInstitute of Pharmacology, Medical University of ViennaInstitute of Pharmacology, Medical University of ViennaInstitute of Pharmacology, Medical University of ViennaKarl Landsteiner Institute for Lung Research and Pulmonary Oncology, Department of Respiratory and Critical Care Medicine, Hospital FloridsdorfInstitute of Pharmacology, Medical University of ViennaAbstract Pleomorphic rhabdomyosarcoma (PRMS) is an extremely rare soft tissue tumor with dismal prognosis that has a higher incidence in adults compared to the other RMS subtypes. The unique PRMS cell line BH1522 was established from a pleural effusion of a lung metastasis and the characteristics of this cell line were compared to two embryonal type RMS (ERMS) cell lines. The affected patient had been treated by surgery, several cycles of chemotherapy and thoracoscopy of the lung metastases. Chemosensitivity of the PRMS cell lines was checked using cytotoxicity assays and oncogene-related and phosphoproteins by Western blot arrays. The BH1522 cell line proved to be chemosensitive to conventional chemotherapeutics and the Son of Sevenless 1 homolog (SOS1) inhibitor BAY-293 that inhibited proliferation and suppressed MYC activity. Results of the novel functional BH1522 tests corroborate published tumor sequencing data that demonstrated alterations in receptor tyrosine kinase/MAPK, PI3K/AKT and mutated p53 as suspected drivers of malignant growth.https://doi.org/10.1038/s41598-025-87027-2Pleomorphic rhabdomyosarcomaCell lineChemosensitivitySOS1MYC |
| spellingShingle | Sandra Stickler Clemens Lang Maximilian Rieche Marie-Therese Eggerstorfer Martin Hohenegger Maximilian Hochmair Gerhard Hamilton Characterization of a pleomorphic rhabdomyosarcoma cell line Scientific Reports Pleomorphic rhabdomyosarcoma Cell line Chemosensitivity SOS1 MYC |
| title | Characterization of a pleomorphic rhabdomyosarcoma cell line |
| title_full | Characterization of a pleomorphic rhabdomyosarcoma cell line |
| title_fullStr | Characterization of a pleomorphic rhabdomyosarcoma cell line |
| title_full_unstemmed | Characterization of a pleomorphic rhabdomyosarcoma cell line |
| title_short | Characterization of a pleomorphic rhabdomyosarcoma cell line |
| title_sort | characterization of a pleomorphic rhabdomyosarcoma cell line |
| topic | Pleomorphic rhabdomyosarcoma Cell line Chemosensitivity SOS1 MYC |
| url | https://doi.org/10.1038/s41598-025-87027-2 |
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