Objective Responses in Metastatic Pleomorphic Rhabdomyosarcoma Treated with Combination of Doxorubicin and Pembrolizumab: A Case Series
Introduction: Pleomorphic rhabdomyosarcoma is a rare subtype of rhabdomyosarcoma, a soft tissue sarcoma with skeletal muscle differentiation. Although rhabdomyosarcoma is typically seen in the pediatric population, the pleomorphic variant most frequently presents in adulthood and is characteristical...
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| Format: | Article |
| Language: | English |
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Karger Publishers
2024-02-01
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| Series: | Case Reports in Oncology |
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| Online Access: | https://beta.karger.com/Article/FullText/535959 |
| _version_ | 1797253813888876544 |
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| author | Ioannis Kournoutas Varun Monga Jonathan Davick John Rieth |
| author_facet | Ioannis Kournoutas Varun Monga Jonathan Davick John Rieth |
| author_sort | Ioannis Kournoutas |
| collection | DOAJ |
| description | Introduction: Pleomorphic rhabdomyosarcoma is a rare subtype of rhabdomyosarcoma, a soft tissue sarcoma with skeletal muscle differentiation. Although rhabdomyosarcoma is typically seen in the pediatric population, the pleomorphic variant most frequently presents in adulthood and is characteristically aggressive with no currently established treatment regimen in the setting of metastatic disease. There has been growing interest in the application of immune checkpoint inhibitors alongside conventional chemotherapeutic agents in the treatment of pleomorphic rhabdomyosarcoma. Case Presentation: In the present case series, we report 2 patients with metastatic pleomorphic rhabdomyosarcoma treated with combination doxorubicin and pembrolizumab who had confirmed objective responses. Of note, these 2 patients had variable PD-L1 status – negative and low positive. Duration of treatment response was notable at 14 months and 9 months, respectively, with the first patient remaining on maintenance pembrolizumab therapy and the second patient subsequently achieving complete response with third-line trabectedin. Both patients are currently undergoing routine interval imaging with no evidence of disease at this time. Conclusion: This report highlights and discusses the potential role of PD-1 blockade in the treatment of pleomorphic rhabdomyosarcoma and also discusses burgeoning immunological data that may explain the clinical responses seen in these 2 cases. |
| first_indexed | 2024-03-07T19:39:12Z |
| format | Article |
| id | doaj.art-fb75268634e8472887885cd1460246fd |
| institution | Directory Open Access Journal |
| issn | 1662-6575 |
| language | English |
| last_indexed | 2024-04-24T21:40:02Z |
| publishDate | 2024-02-01 |
| publisher | Karger Publishers |
| record_format | Article |
| series | Case Reports in Oncology |
| spelling | doaj.art-fb75268634e8472887885cd1460246fd2024-03-21T08:27:29ZengKarger PublishersCase Reports in Oncology1662-65752024-02-0117134435110.1159/000535959535959Objective Responses in Metastatic Pleomorphic Rhabdomyosarcoma Treated with Combination of Doxorubicin and Pembrolizumab: A Case SeriesIoannis Kournoutas0Varun Monga1Jonathan Davick2John Rieth3Department of Internal Medicine, University of Iowa Carver College of Medicine, Iowa City, IA, USADivision of Hematology, Oncology, and Blood and Marrow Transplantation, Department of Internal Medicine, University of Iowa, Iowa City, IA, USADepartment of Pathology, University of Iowa, Iowa City, IA, USADivision of Hematology, Oncology, and Blood and Marrow Transplantation, Department of Internal Medicine, University of Iowa, Iowa City, IA, USAIntroduction: Pleomorphic rhabdomyosarcoma is a rare subtype of rhabdomyosarcoma, a soft tissue sarcoma with skeletal muscle differentiation. Although rhabdomyosarcoma is typically seen in the pediatric population, the pleomorphic variant most frequently presents in adulthood and is characteristically aggressive with no currently established treatment regimen in the setting of metastatic disease. There has been growing interest in the application of immune checkpoint inhibitors alongside conventional chemotherapeutic agents in the treatment of pleomorphic rhabdomyosarcoma. Case Presentation: In the present case series, we report 2 patients with metastatic pleomorphic rhabdomyosarcoma treated with combination doxorubicin and pembrolizumab who had confirmed objective responses. Of note, these 2 patients had variable PD-L1 status – negative and low positive. Duration of treatment response was notable at 14 months and 9 months, respectively, with the first patient remaining on maintenance pembrolizumab therapy and the second patient subsequently achieving complete response with third-line trabectedin. Both patients are currently undergoing routine interval imaging with no evidence of disease at this time. Conclusion: This report highlights and discusses the potential role of PD-1 blockade in the treatment of pleomorphic rhabdomyosarcoma and also discusses burgeoning immunological data that may explain the clinical responses seen in these 2 cases.https://beta.karger.com/Article/FullText/535959rhabdomyosarcomapleomorphic rhabdomyosarcomaimmune checkpointpembrolizumabdoxorubicincase series |
| spellingShingle | Ioannis Kournoutas Varun Monga Jonathan Davick John Rieth Objective Responses in Metastatic Pleomorphic Rhabdomyosarcoma Treated with Combination of Doxorubicin and Pembrolizumab: A Case Series Case Reports in Oncology rhabdomyosarcoma pleomorphic rhabdomyosarcoma immune checkpoint pembrolizumab doxorubicin case series |
| title | Objective Responses in Metastatic Pleomorphic Rhabdomyosarcoma Treated with Combination of Doxorubicin and Pembrolizumab: A Case Series |
| title_full | Objective Responses in Metastatic Pleomorphic Rhabdomyosarcoma Treated with Combination of Doxorubicin and Pembrolizumab: A Case Series |
| title_fullStr | Objective Responses in Metastatic Pleomorphic Rhabdomyosarcoma Treated with Combination of Doxorubicin and Pembrolizumab: A Case Series |
| title_full_unstemmed | Objective Responses in Metastatic Pleomorphic Rhabdomyosarcoma Treated with Combination of Doxorubicin and Pembrolizumab: A Case Series |
| title_short | Objective Responses in Metastatic Pleomorphic Rhabdomyosarcoma Treated with Combination of Doxorubicin and Pembrolizumab: A Case Series |
| title_sort | objective responses in metastatic pleomorphic rhabdomyosarcoma treated with combination of doxorubicin and pembrolizumab a case series |
| topic | rhabdomyosarcoma pleomorphic rhabdomyosarcoma immune checkpoint pembrolizumab doxorubicin case series |
| url | https://beta.karger.com/Article/FullText/535959 |
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