A Retrospective Multi-Institutional Cohort Analysis of Clinical Characteristics and Outcomes in Dedifferentiated Chondrosarcoma
Background: Dedifferentiated chondrosarcoma (DDCS) is a rare subset of chondrosarcoma. It is an aggressive neoplasm characterized by a high rate of recurrent and metastatic disease with overall poor outcomes. Systemic therapy is often used to treat DDCS; however, the optimal regimen and timing are n...
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| Format: | Article |
| Language: | English |
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MDPI AG
2023-05-01
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| Series: | Cancers |
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| Online Access: | https://www.mdpi.com/2072-6694/15/9/2617 |
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| author | Nam Bui Hilary Dietz Sheima Farag Angela C. Hirbe Michael J. Wagner Brian A. Van Tine Kristen Ganjoo Robin L. Jones Vicki L. Keedy Elizabeth J. Davis |
| author_facet | Nam Bui Hilary Dietz Sheima Farag Angela C. Hirbe Michael J. Wagner Brian A. Van Tine Kristen Ganjoo Robin L. Jones Vicki L. Keedy Elizabeth J. Davis |
| author_sort | Nam Bui |
| collection | DOAJ |
| description | Background: Dedifferentiated chondrosarcoma (DDCS) is a rare subset of chondrosarcoma. It is an aggressive neoplasm characterized by a high rate of recurrent and metastatic disease with overall poor outcomes. Systemic therapy is often used to treat DDCS; however, the optimal regimen and timing are not well defined, with current guidelines recommending following osteosarcoma protocols. Methods: We conducted a multi-institutional retrospective analysis of clinical characteristics and outcomes of patients with DDCS. Between 1 January 2004 and 1 January 2022, the databases from five academic sarcoma centers were reviewed. Patient and tumor factors, including age, sex, tumor size, site, location, the treatments rendered, and survival outcomes, were collected. Results: Seventy-four patients were identified and included in the analysis. Most patients presented with localized disease. Surgical resection was the mainstay of therapy. Chemotherapy was used predominantly in the metastatic setting. Partial responses were low (n = 4; 9%) and occurred upon treatment with doxorubicin with cisplatin or ifosfamide and single-agent pembrolizumab. For all other regimens, stable disease was the best response. Prolonged stable disease occurred with the use of pazopanib and immune checkpoint inhibitors. Conclusions: DDCS has poor outcomes and conventional chemotherapy has limited benefit. Future studies should focus on defining the possible role of molecularly targeted therapies and immunotherapy in the treatment of DDCS. |
| first_indexed | 2024-03-11T04:21:53Z |
| format | Article |
| id | doaj.art-2010fc00c1874739b50e2dcd953f7d87 |
| institution | Directory Open Access Journal |
| issn | 2072-6694 |
| language | English |
| last_indexed | 2024-03-11T04:21:53Z |
| publishDate | 2023-05-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Cancers |
| spelling | doaj.art-2010fc00c1874739b50e2dcd953f7d872023-11-17T22:42:14ZengMDPI AGCancers2072-66942023-05-01159261710.3390/cancers15092617A Retrospective Multi-Institutional Cohort Analysis of Clinical Characteristics and Outcomes in Dedifferentiated ChondrosarcomaNam Bui0Hilary Dietz1Sheima Farag2Angela C. Hirbe3Michael J. Wagner4Brian A. Van Tine5Kristen Ganjoo6Robin L. Jones7Vicki L. Keedy8Elizabeth J. Davis9Stanford Cancer Institute, Palo Alto, CA 94304, USAVanderbilt University Medical Center, Nashville, TN 37232, USAInstitute of Cancer Research, Royal Marsden Hospital, London SW3 6JJ, UKSiteman Cancer Center, Washington University in St. Louis, St. Louis, MO 63110, USAFred Hutchinson Cancer Center, University of Washington, Seattle, WA 98109, USASiteman Cancer Center, Washington University in St. Louis, St. Louis, MO 63110, USAStanford Cancer Institute, Palo Alto, CA 94304, USAInstitute of Cancer Research, Royal Marsden Hospital, London SW3 6JJ, UKVanderbilt University Medical Center, Nashville, TN 37232, USAVanderbilt University Medical Center, Nashville, TN 37232, USABackground: Dedifferentiated chondrosarcoma (DDCS) is a rare subset of chondrosarcoma. It is an aggressive neoplasm characterized by a high rate of recurrent and metastatic disease with overall poor outcomes. Systemic therapy is often used to treat DDCS; however, the optimal regimen and timing are not well defined, with current guidelines recommending following osteosarcoma protocols. Methods: We conducted a multi-institutional retrospective analysis of clinical characteristics and outcomes of patients with DDCS. Between 1 January 2004 and 1 January 2022, the databases from five academic sarcoma centers were reviewed. Patient and tumor factors, including age, sex, tumor size, site, location, the treatments rendered, and survival outcomes, were collected. Results: Seventy-four patients were identified and included in the analysis. Most patients presented with localized disease. Surgical resection was the mainstay of therapy. Chemotherapy was used predominantly in the metastatic setting. Partial responses were low (n = 4; 9%) and occurred upon treatment with doxorubicin with cisplatin or ifosfamide and single-agent pembrolizumab. For all other regimens, stable disease was the best response. Prolonged stable disease occurred with the use of pazopanib and immune checkpoint inhibitors. Conclusions: DDCS has poor outcomes and conventional chemotherapy has limited benefit. Future studies should focus on defining the possible role of molecularly targeted therapies and immunotherapy in the treatment of DDCS.https://www.mdpi.com/2072-6694/15/9/2617sarcomabone sarcomachondrosarcomadedifferentiated chondrosarcoma |
| spellingShingle | Nam Bui Hilary Dietz Sheima Farag Angela C. Hirbe Michael J. Wagner Brian A. Van Tine Kristen Ganjoo Robin L. Jones Vicki L. Keedy Elizabeth J. Davis A Retrospective Multi-Institutional Cohort Analysis of Clinical Characteristics and Outcomes in Dedifferentiated Chondrosarcoma Cancers sarcoma bone sarcoma chondrosarcoma dedifferentiated chondrosarcoma |
| title | A Retrospective Multi-Institutional Cohort Analysis of Clinical Characteristics and Outcomes in Dedifferentiated Chondrosarcoma |
| title_full | A Retrospective Multi-Institutional Cohort Analysis of Clinical Characteristics and Outcomes in Dedifferentiated Chondrosarcoma |
| title_fullStr | A Retrospective Multi-Institutional Cohort Analysis of Clinical Characteristics and Outcomes in Dedifferentiated Chondrosarcoma |
| title_full_unstemmed | A Retrospective Multi-Institutional Cohort Analysis of Clinical Characteristics and Outcomes in Dedifferentiated Chondrosarcoma |
| title_short | A Retrospective Multi-Institutional Cohort Analysis of Clinical Characteristics and Outcomes in Dedifferentiated Chondrosarcoma |
| title_sort | retrospective multi institutional cohort analysis of clinical characteristics and outcomes in dedifferentiated chondrosarcoma |
| topic | sarcoma bone sarcoma chondrosarcoma dedifferentiated chondrosarcoma |
| url | https://www.mdpi.com/2072-6694/15/9/2617 |
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