Primary cardiac sarcomas: A multi‐national retrospective review
Background Primary cardiac sarcoma (PCS) is a rare but often fatal disease. The current study aimed to analyze the impact of baseline demographics, local and systemic therapies in a contemporary cohort. Methods Clinical records of PCS across six institutions in three continents were reviewed. Kaplan...
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| Veröffentlicht in: | Cancer medicine (Malden, MA) MA), 2019-01, Vol.8 (1), p.104-110 |
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| creator | Chen, Tom Wei‐Wu Loong, Herbert H. Srikanthan, Amirrtha Zer, Alona Barua, Reeta Butany, Jagdish Cusimano, Robert J. Liang, Yun‐Chieh Chang, Chin‐Hao Iakobishvili, Zaza Razak, Albiruni R. Abdul Lewin, Jeremy |
| description | Background
Primary cardiac sarcoma (PCS) is a rare but often fatal disease. The current study aimed to analyze the impact of baseline demographics, local and systemic therapies in a contemporary cohort.
Methods
Clinical records of PCS across six institutions in three continents were reviewed. Kaplan‐Meier method was used to estimate survival. Cox proportional hazard model was used to determine variables impacting progression‐free survival (PFS) or overall survival (OS).
Results
Sixty‐one patients with PCS (1996‐2016) were identified. The median age at diagnosis was 46 (range 18‐79); 36% (n = 22) presented with metastatic disease. The most common histology was angiosarcoma (n = 24, 39%). A total of 46 patients received surgery (75%) but only 5 (8%) patients achieved R0 resection. Multi‐modality treatment to the primary tumor was given to 28 patients (46%; localized disease 23/39 (59%); metastatic disease 5/22 (23%)). The median OS for the entire cohort was 17.5 months (95% CI 9.5‐20.6), with seven (11%) patients surviving longer than 36 months. On multi‐variate analysis, age |
| doi_str_mv | 10.1002/cam4.1897 |
| format | Article |
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Primary cardiac sarcoma (PCS) is a rare but often fatal disease. The current study aimed to analyze the impact of baseline demographics, local and systemic therapies in a contemporary cohort.
Methods
Clinical records of PCS across six institutions in three continents were reviewed. Kaplan‐Meier method was used to estimate survival. Cox proportional hazard model was used to determine variables impacting progression‐free survival (PFS) or overall survival (OS).
Results
Sixty‐one patients with PCS (1996‐2016) were identified. The median age at diagnosis was 46 (range 18‐79); 36% (n = 22) presented with metastatic disease. The most common histology was angiosarcoma (n = 24, 39%). A total of 46 patients received surgery (75%) but only 5 (8%) patients achieved R0 resection. Multi‐modality treatment to the primary tumor was given to 28 patients (46%; localized disease 23/39 (59%); metastatic disease 5/22 (23%)). The median OS for the entire cohort was 17.5 months (95% CI 9.5‐20.6), with seven (11%) patients surviving longer than 36 months. On multi‐variate analysis, age <65 (P = 0.01) was the only significant favorable prognostic factor. For first‐line palliative chemotherapy, the median PFS was 4.4 months (95% CI 2.9‐7.7 months). The best response for first‐line chemotherapy was 32% (CR = 1, PR = 9). No significant improvement in OS was identified in patients presenting throughout the 20‐year period of this review.
Conclusion
Younger age at diagnosis was associated with improved outcome although the prognosis of PCS remains poor. Given the lack of improvement in survival, further dedicated research is required.
Primary cardiac sarcoma (PCS) is a rare disease with poor prognosis. The survival had not improved in the past 20 years. Identification of prognostic factors and better treatment modalities to PCD is crucial to improve the outcome of PCS patients.</description><identifier>ISSN: 2045-7634</identifier><identifier>EISSN: 2045-7634</identifier><identifier>DOI: 10.1002/cam4.1897</identifier><identifier>PMID: 30575309</identifier><language>eng</language><publisher>United States: John Wiley & Sons, Inc</publisher><subject>Adolescent ; Adult ; Age ; Aged ; Chemotherapy ; Clinical Cancer Research ; Demography ; Diagnosis ; Female ; Heart Neoplasms - diagnosis ; Heart Neoplasms - epidemiology ; Heart Neoplasms - pathology ; Heart Neoplasms - therapy ; Humans ; Male ; Medical prognosis ; Metastases ; Metastasis ; Middle Aged ; Original Research ; Patients ; primary cardiac sarcoma ; radiotherapy ; Retrospective Studies ; Sarcoma ; Sarcoma - diagnosis ; Sarcoma - epidemiology ; Sarcoma - pathology ; Sarcoma - therapy ; sarcoma chemotherapy ; Surgery ; Survival ; Survival Analysis ; Treatment Outcome ; Tumor Burden ; Young Adult</subject><ispartof>Cancer medicine (Malden, MA), 2019-01, Vol.8 (1), p.104-110</ispartof><rights>2018 The Authors. published by John Wiley & Sons Ltd.</rights><rights>2018 The Authors. Cancer Medicine published by John Wiley & Sons Ltd.</rights><rights>2019. This work is published under http://creativecommons.org/licenses/by/4.0/ (the “License”). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c5097-ec9fefbd75f8ec1bf2957dd474a062c58ccab2d681f6f331907393b6ba02d9e63</citedby><cites>FETCH-LOGICAL-c5097-ec9fefbd75f8ec1bf2957dd474a062c58ccab2d681f6f331907393b6ba02d9e63</cites><orcidid>0000-0003-4112-4029</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC6346258/pdf/$$EPDF$$P50$$Gpubmedcentral$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC6346258/$$EHTML$$P50$$Gpubmedcentral$$Hfree_for_read</linktohtml><link.rule.ids>230,314,723,776,780,860,881,1411,11542,27903,27904,45553,45554,46030,46454,53769,53771</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/30575309$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Chen, Tom Wei‐Wu</creatorcontrib><creatorcontrib>Loong, Herbert H.</creatorcontrib><creatorcontrib>Srikanthan, Amirrtha</creatorcontrib><creatorcontrib>Zer, Alona</creatorcontrib><creatorcontrib>Barua, Reeta</creatorcontrib><creatorcontrib>Butany, Jagdish</creatorcontrib><creatorcontrib>Cusimano, Robert J.</creatorcontrib><creatorcontrib>Liang, Yun‐Chieh</creatorcontrib><creatorcontrib>Chang, Chin‐Hao</creatorcontrib><creatorcontrib>Iakobishvili, Zaza</creatorcontrib><creatorcontrib>Razak, Albiruni R. Abdul</creatorcontrib><creatorcontrib>Lewin, Jeremy</creatorcontrib><title>Primary cardiac sarcomas: A multi‐national retrospective review</title><title>Cancer medicine (Malden, MA)</title><addtitle>Cancer Med</addtitle><description>Background
Primary cardiac sarcoma (PCS) is a rare but often fatal disease. The current study aimed to analyze the impact of baseline demographics, local and systemic therapies in a contemporary cohort.
Methods
Clinical records of PCS across six institutions in three continents were reviewed. Kaplan‐Meier method was used to estimate survival. Cox proportional hazard model was used to determine variables impacting progression‐free survival (PFS) or overall survival (OS).
Results
Sixty‐one patients with PCS (1996‐2016) were identified. The median age at diagnosis was 46 (range 18‐79); 36% (n = 22) presented with metastatic disease. The most common histology was angiosarcoma (n = 24, 39%). A total of 46 patients received surgery (75%) but only 5 (8%) patients achieved R0 resection. Multi‐modality treatment to the primary tumor was given to 28 patients (46%; localized disease 23/39 (59%); metastatic disease 5/22 (23%)). The median OS for the entire cohort was 17.5 months (95% CI 9.5‐20.6), with seven (11%) patients surviving longer than 36 months. On multi‐variate analysis, age <65 (P = 0.01) was the only significant favorable prognostic factor. For first‐line palliative chemotherapy, the median PFS was 4.4 months (95% CI 2.9‐7.7 months). The best response for first‐line chemotherapy was 32% (CR = 1, PR = 9). No significant improvement in OS was identified in patients presenting throughout the 20‐year period of this review.
Conclusion
Younger age at diagnosis was associated with improved outcome although the prognosis of PCS remains poor. Given the lack of improvement in survival, further dedicated research is required.
Primary cardiac sarcoma (PCS) is a rare disease with poor prognosis. The survival had not improved in the past 20 years. Identification of prognostic factors and better treatment modalities to PCD is crucial to improve the outcome of PCS patients.</description><subject>Adolescent</subject><subject>Adult</subject><subject>Age</subject><subject>Aged</subject><subject>Chemotherapy</subject><subject>Clinical Cancer Research</subject><subject>Demography</subject><subject>Diagnosis</subject><subject>Female</subject><subject>Heart Neoplasms - diagnosis</subject><subject>Heart Neoplasms - epidemiology</subject><subject>Heart Neoplasms - pathology</subject><subject>Heart Neoplasms - therapy</subject><subject>Humans</subject><subject>Male</subject><subject>Medical prognosis</subject><subject>Metastases</subject><subject>Metastasis</subject><subject>Middle Aged</subject><subject>Original Research</subject><subject>Patients</subject><subject>primary cardiac sarcoma</subject><subject>radiotherapy</subject><subject>Retrospective Studies</subject><subject>Sarcoma</subject><subject>Sarcoma - diagnosis</subject><subject>Sarcoma - epidemiology</subject><subject>Sarcoma - pathology</subject><subject>Sarcoma - therapy</subject><subject>sarcoma chemotherapy</subject><subject>Surgery</subject><subject>Survival</subject><subject>Survival Analysis</subject><subject>Treatment Outcome</subject><subject>Tumor Burden</subject><subject>Young Adult</subject><issn>2045-7634</issn><issn>2045-7634</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2019</creationdate><recordtype>article</recordtype><sourceid>24P</sourceid><sourceid>EIF</sourceid><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>AZQEC</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><sourceid>DWQXO</sourceid><sourceid>GNUQQ</sourceid><recordid>eNp1kctKAzEYhYMottQufAEZcKOLtrlMJhMXQineoKILXYdMJqMpc6nJTEt3PoLP6JOYsbVUwX-T_ycfh8M5ABwjOEQQ4pGSRThEMWd7oIthSAcsIuH-zt4Bfedm0A-DOGLoEHQIpIwSyLtg_GhNIe0qUNKmRqrASauqQrqLYBwUTV6bz_ePUtamKmUeWF3bys21qs1C-2th9PIIHGQyd7q_eXvg-frqaXI7mD7c3E3G04GikLOBVjzTWZIymsVaoSTDnLI0DVkoYYQVjZWSCU6jGGVRRgjikBFOkiiREKdcR6QHLte68yYpdKp0WVuZi_nav6ikEb9_SvMqXqqF8BFEmMZe4GwjYKu3RrtaFMYpneey1FXjBEaU8xhxzj16-gedVY31CbQUgzEhJG4dna8p5UNxVmdbMwiKthvRdiPabjx7sut-S_404YHRGliaXK_-VxKT8X34LfkFBwqaEw</recordid><startdate>201901</startdate><enddate>201901</enddate><creator>Chen, Tom Wei‐Wu</creator><creator>Loong, Herbert H.</creator><creator>Srikanthan, Amirrtha</creator><creator>Zer, Alona</creator><creator>Barua, Reeta</creator><creator>Butany, Jagdish</creator><creator>Cusimano, Robert J.</creator><creator>Liang, Yun‐Chieh</creator><creator>Chang, Chin‐Hao</creator><creator>Iakobishvili, Zaza</creator><creator>Razak, Albiruni R. Abdul</creator><creator>Lewin, Jeremy</creator><general>John Wiley & Sons, Inc</general><general>John Wiley and Sons Inc</general><scope>24P</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>3V.</scope><scope>7X7</scope><scope>7XB</scope><scope>8FE</scope><scope>8FH</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>AZQEC</scope><scope>BBNVY</scope><scope>BENPR</scope><scope>BHPHI</scope><scope>CCPQU</scope><scope>DWQXO</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>GNUQQ</scope><scope>HCIFZ</scope><scope>K9.</scope><scope>LK8</scope><scope>M0S</scope><scope>M7P</scope><scope>PIMPY</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>7X8</scope><scope>5PM</scope><orcidid>https://orcid.org/0000-0003-4112-4029</orcidid></search><sort><creationdate>201901</creationdate><title>Primary cardiac sarcomas: A multi‐national retrospective review</title><author>Chen, Tom Wei‐Wu ; Loong, Herbert H. ; Srikanthan, Amirrtha ; Zer, Alona ; Barua, Reeta ; Butany, Jagdish ; Cusimano, Robert J. ; Liang, Yun‐Chieh ; Chang, Chin‐Hao ; Iakobishvili, Zaza ; Razak, Albiruni R. Abdul ; Lewin, Jeremy</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c5097-ec9fefbd75f8ec1bf2957dd474a062c58ccab2d681f6f331907393b6ba02d9e63</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2019</creationdate><topic>Adolescent</topic><topic>Adult</topic><topic>Age</topic><topic>Aged</topic><topic>Chemotherapy</topic><topic>Clinical Cancer Research</topic><topic>Demography</topic><topic>Diagnosis</topic><topic>Female</topic><topic>Heart Neoplasms - diagnosis</topic><topic>Heart Neoplasms - epidemiology</topic><topic>Heart Neoplasms - pathology</topic><topic>Heart Neoplasms - therapy</topic><topic>Humans</topic><topic>Male</topic><topic>Medical prognosis</topic><topic>Metastases</topic><topic>Metastasis</topic><topic>Middle Aged</topic><topic>Original Research</topic><topic>Patients</topic><topic>primary cardiac sarcoma</topic><topic>radiotherapy</topic><topic>Retrospective Studies</topic><topic>Sarcoma</topic><topic>Sarcoma - diagnosis</topic><topic>Sarcoma - epidemiology</topic><topic>Sarcoma - pathology</topic><topic>Sarcoma - therapy</topic><topic>sarcoma chemotherapy</topic><topic>Surgery</topic><topic>Survival</topic><topic>Survival Analysis</topic><topic>Treatment Outcome</topic><topic>Tumor Burden</topic><topic>Young Adult</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Chen, Tom Wei‐Wu</creatorcontrib><creatorcontrib>Loong, Herbert H.</creatorcontrib><creatorcontrib>Srikanthan, Amirrtha</creatorcontrib><creatorcontrib>Zer, Alona</creatorcontrib><creatorcontrib>Barua, Reeta</creatorcontrib><creatorcontrib>Butany, Jagdish</creatorcontrib><creatorcontrib>Cusimano, Robert J.</creatorcontrib><creatorcontrib>Liang, Yun‐Chieh</creatorcontrib><creatorcontrib>Chang, Chin‐Hao</creatorcontrib><creatorcontrib>Iakobishvili, Zaza</creatorcontrib><creatorcontrib>Razak, Albiruni R. Abdul</creatorcontrib><creatorcontrib>Lewin, Jeremy</creatorcontrib><collection>Wiley Online Library Open Access</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>ProQuest SciTech Collection</collection><collection>ProQuest Natural Science Collection</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>ProQuest Central (Alumni Edition)</collection><collection>ProQuest Central UK/Ireland</collection><collection>ProQuest Central Essentials</collection><collection>Biological Science Collection</collection><collection>ProQuest Central</collection><collection>Natural Science Collection</collection><collection>ProQuest One Community College</collection><collection>ProQuest Central Korea</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>ProQuest Central Student</collection><collection>SciTech Premium Collection</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>ProQuest Biological Science Collection</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Biological Science Database</collection><collection>Publicly Available Content Database</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>MEDLINE - Academic</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>Cancer medicine (Malden, MA)</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Chen, Tom Wei‐Wu</au><au>Loong, Herbert H.</au><au>Srikanthan, Amirrtha</au><au>Zer, Alona</au><au>Barua, Reeta</au><au>Butany, Jagdish</au><au>Cusimano, Robert J.</au><au>Liang, Yun‐Chieh</au><au>Chang, Chin‐Hao</au><au>Iakobishvili, Zaza</au><au>Razak, Albiruni R. Abdul</au><au>Lewin, Jeremy</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Primary cardiac sarcomas: A multi‐national retrospective review</atitle><jtitle>Cancer medicine (Malden, MA)</jtitle><addtitle>Cancer Med</addtitle><date>2019-01</date><risdate>2019</risdate><volume>8</volume><issue>1</issue><spage>104</spage><epage>110</epage><pages>104-110</pages><issn>2045-7634</issn><eissn>2045-7634</eissn><abstract>Background
Primary cardiac sarcoma (PCS) is a rare but often fatal disease. The current study aimed to analyze the impact of baseline demographics, local and systemic therapies in a contemporary cohort.
Methods
Clinical records of PCS across six institutions in three continents were reviewed. Kaplan‐Meier method was used to estimate survival. Cox proportional hazard model was used to determine variables impacting progression‐free survival (PFS) or overall survival (OS).
Results
Sixty‐one patients with PCS (1996‐2016) were identified. The median age at diagnosis was 46 (range 18‐79); 36% (n = 22) presented with metastatic disease. The most common histology was angiosarcoma (n = 24, 39%). A total of 46 patients received surgery (75%) but only 5 (8%) patients achieved R0 resection. Multi‐modality treatment to the primary tumor was given to 28 patients (46%; localized disease 23/39 (59%); metastatic disease 5/22 (23%)). The median OS for the entire cohort was 17.5 months (95% CI 9.5‐20.6), with seven (11%) patients surviving longer than 36 months. On multi‐variate analysis, age <65 (P = 0.01) was the only significant favorable prognostic factor. For first‐line palliative chemotherapy, the median PFS was 4.4 months (95% CI 2.9‐7.7 months). The best response for first‐line chemotherapy was 32% (CR = 1, PR = 9). No significant improvement in OS was identified in patients presenting throughout the 20‐year period of this review.
Conclusion
Younger age at diagnosis was associated with improved outcome although the prognosis of PCS remains poor. Given the lack of improvement in survival, further dedicated research is required.
Primary cardiac sarcoma (PCS) is a rare disease with poor prognosis. The survival had not improved in the past 20 years. Identification of prognostic factors and better treatment modalities to PCD is crucial to improve the outcome of PCS patients.</abstract><cop>United States</cop><pub>John Wiley & Sons, Inc</pub><pmid>30575309</pmid><doi>10.1002/cam4.1897</doi><tpages>7</tpages><orcidid>https://orcid.org/0000-0003-4112-4029</orcidid><oa>free_for_read</oa></addata></record> |
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| source | MEDLINE; DOAJ Directory of Open Access Journals; Wiley Online Library Journals Frontfile Complete; Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals; Wiley Online Library Open Access; PubMed Central |
| subjects | Adolescent Adult Age Aged Chemotherapy Clinical Cancer Research Demography Diagnosis Female Heart Neoplasms - diagnosis Heart Neoplasms - epidemiology Heart Neoplasms - pathology Heart Neoplasms - therapy Humans Male Medical prognosis Metastases Metastasis Middle Aged Original Research Patients primary cardiac sarcoma radiotherapy Retrospective Studies Sarcoma Sarcoma - diagnosis Sarcoma - epidemiology Sarcoma - pathology Sarcoma - therapy sarcoma chemotherapy Surgery Survival Survival Analysis Treatment Outcome Tumor Burden Young Adult |
| title | Primary cardiac sarcomas: A multi‐national retrospective review |
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