Clinical Outcomes Among Immunotherapy-Treated Patients With Primary Cardiac Soft Tissue Sarcomas: A Multicenter Retrospective Study

Amin H. Nassar; Edward El-Am; Ryan Denu; Sarah Abou Alaiwi; Talal El Zarif; Walid Macaron; Noha Abdel-Wahab; Aakash Desai; Caleb Smith; Kaushal Parikh; Muhannad Abbasi; Elias Bou Farhat; James M. Williams; Jeremy D. Collins; Ahmad Al-Hader; Rana R. McKay; Carmel Malvar; Mohamad Sabra; Caiwei Zhong; Raquelle El Alam; Omar Chehab; Joao Lima; Minh Phan; Hanna Ferreira Dalla Pria; Alexandra Trevino; Tomas G. Neilan; Jennifer M. Kwan; Vinod Ravi; Hari Deshpande; George Demetri; Toni K. Choueiri; Abdul Rafeh Naqash

doi:10.1016/j.jaccao.2023.11.007

Clinical Outcomes Among Immunotherapy-Treated Patients With Primary Cardiac Soft Tissue Sarcomas: A Multicenter Retrospective Study

Amin H. Nassar, Edward El-Am, Ryan Denu, Sarah Abou Alaiwi, Talal El Zarif, Walid Macaron, Noha Abdel-Wahab, Aakash Desai, Caleb Smith, Kaushal Parikh, Muhannad Abbasi, Elias Bou Farhat, James M. Williams, Jeremy D. Collins, Ahmad Al-Hader, Rana R. McKay, Carmel Malvar, Mohamad Sabra, Caiwei Zhong, Raquelle El AlamOmar Chehab, Joao Lima, Minh Phan, Hanna Ferreira Dalla Pria, Alexandra Trevino, Tomas G. Neilan, Jennifer M. Kwan, Vinod Ravi, Hari Deshpande, George Demetri, Toni K. Choueiri, Abdul Rafeh Naqash

Research output: Contribution to journal › Article › peer-review

1 Scopus citations

Abstract

Background: Primary cardiac soft tissue sarcomas (CSTS) affect young adults, with dismal outcomes. Objectives: The aim of this study was to investigate the clinical outcomes of patients with CSTS receiving immune checkpoint inhibitors (ICIs). Methods: A retrospective, multi-institutional cohort study was conducted among patients with CSTS between 2015 and 2022. The patients were treated with ICI-based regimens. The Kaplan-Meier method was used to estimate overall survival (OS) and progression-free survival (PFS). Objective response rates were determined according to Response Evaluation Criteria in Solid Tumors version 1.1. Treatment-related adverse events were graded per the Common Terminology Criteria for Adverse Events version 5.0. Results: Among 24 patients with CSTS, 17 (70.8%) were White, and 13 (54.2%) were male. Eight patients (33.3%) had angiosarcoma. At the time of ICI treatment, 18 patients (75.0%) had metastatic CSTS, and 4 (16.7%) had locally advanced disease. ICIs were administered as the first-line therapy in 6 patients (25.0%) and as the second-line therapy or beyond in 18 patients (75.0%). For the 18 patients with available response data, objective response rate was 11.1% (n = 2 of 18). The median PFS and median OS in advanced and metastatic CSTS (n = 22) were 5.7 months (95% CI: 2.8-13.3 months) and 14.9 months (95% CI: 5.7-23.7 months), respectively. The median PFS and OS were significantly shorter in patients with cardiac angiosarcomas than in those with nonangiosarcoma CSTS: median PFS was 1.7 vs 11 months, respectively (P < 0.0001), and median OS was 3.0 vs 24.0 months, respectively (P = 0.008). Any grade treatment-related adverse events occurred exclusively in the 15 patients with nonangiosarcoma CSTS (n = 7 [46.7%]), of which 6 (40.0%) were grade ≥3. Conclusions: Although ICIs demonstrate modest activity in CSTS, durable benefit was observed in a subset of patients with nonangiosarcoma, albeit with higher toxicity.

Original language	English (US)
Pages (from-to)	71-79
Number of pages	9
Journal	JACC: CardioOncology
Volume	6
Issue number	1
DOIs	https://doi.org/10.1016/j.jaccao.2023.11.007
State	Published - Feb 2024

Keywords

cardiac sarcomas
cardiac tumors
immune checkpoint inhibitors
treatment-related adverse events

ASJC Scopus subject areas

Oncology
Cardiology and Cardiovascular Medicine

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10.1016/j.jaccao.2023.11.007

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Nassar, A. H., El-Am, E., Denu, R., Abou Alaiwi, S., El Zarif, T., Macaron, W., Abdel-Wahab, N., Desai, A., Smith, C., Parikh, K., Abbasi, M., Bou Farhat, E., Williams, J. M., Collins, J. D., Al-Hader, A., McKay, R. R., Malvar, C., Sabra, M., Zhong, C., ... Naqash, A. R. (2024). Clinical Outcomes Among Immunotherapy-Treated Patients With Primary Cardiac Soft Tissue Sarcomas: A Multicenter Retrospective Study. JACC: CardioOncology, 6(1), 71-79. https://doi.org/10.1016/j.jaccao.2023.11.007

Nassar, AH, El-Am, E, Denu, R, Abou Alaiwi, S, El Zarif, T, Macaron, W, Abdel-Wahab, N, Desai, A, Smith, C, Parikh, K, Abbasi, M, Bou Farhat, E, Williams, JM, Collins, JD, Al-Hader, A, McKay, RR, Malvar, C, Sabra, M, Zhong, C, El Alam, R, Chehab, O, Lima, J, Phan, M, Dalla Pria, HF, Trevino, A, Neilan, TG, Kwan, JM, Ravi, V, Deshpande, H, Demetri, G, Choueiri, TK & Naqash, AR 2024, 'Clinical Outcomes Among Immunotherapy-Treated Patients With Primary Cardiac Soft Tissue Sarcomas: A Multicenter Retrospective Study', JACC: CardioOncology, vol. 6, no. 1, pp. 71-79. https://doi.org/10.1016/j.jaccao.2023.11.007

@article{5368a94dff35436eaefa85b10a1a763b,

title = "Clinical Outcomes Among Immunotherapy-Treated Patients With Primary Cardiac Soft Tissue Sarcomas: A Multicenter Retrospective Study",

abstract = "Background: Primary cardiac soft tissue sarcomas (CSTS) affect young adults, with dismal outcomes. Objectives: The aim of this study was to investigate the clinical outcomes of patients with CSTS receiving immune checkpoint inhibitors (ICIs). Methods: A retrospective, multi-institutional cohort study was conducted among patients with CSTS between 2015 and 2022. The patients were treated with ICI-based regimens. The Kaplan-Meier method was used to estimate overall survival (OS) and progression-free survival (PFS). Objective response rates were determined according to Response Evaluation Criteria in Solid Tumors version 1.1. Treatment-related adverse events were graded per the Common Terminology Criteria for Adverse Events version 5.0. Results: Among 24 patients with CSTS, 17 (70.8%) were White, and 13 (54.2%) were male. Eight patients (33.3%) had angiosarcoma. At the time of ICI treatment, 18 patients (75.0%) had metastatic CSTS, and 4 (16.7%) had locally advanced disease. ICIs were administered as the first-line therapy in 6 patients (25.0%) and as the second-line therapy or beyond in 18 patients (75.0%). For the 18 patients with available response data, objective response rate was 11.1% (n = 2 of 18). The median PFS and median OS in advanced and metastatic CSTS (n = 22) were 5.7 months (95% CI: 2.8-13.3 months) and 14.9 months (95% CI: 5.7-23.7 months), respectively. The median PFS and OS were significantly shorter in patients with cardiac angiosarcomas than in those with nonangiosarcoma CSTS: median PFS was 1.7 vs 11 months, respectively (P < 0.0001), and median OS was 3.0 vs 24.0 months, respectively (P = 0.008). Any grade treatment-related adverse events occurred exclusively in the 15 patients with nonangiosarcoma CSTS (n = 7 [46.7%]), of which 6 (40.0%) were grade ≥3. Conclusions: Although ICIs demonstrate modest activity in CSTS, durable benefit was observed in a subset of patients with nonangiosarcoma, albeit with higher toxicity.",

keywords = "cardiac sarcomas, cardiac tumors, immune checkpoint inhibitors, treatment-related adverse events",

author = "Nassar, {Amin H.} and Edward El-Am and Ryan Denu and {Abou Alaiwi}, Sarah and {El Zarif}, Talal and Walid Macaron and Noha Abdel-Wahab and Aakash Desai and Caleb Smith and Kaushal Parikh and Muhannad Abbasi and {Bou Farhat}, Elias and Williams, {James M.} and Collins, {Jeremy D.} and Ahmad Al-Hader and McKay, {Rana R.} and Carmel Malvar and Mohamad Sabra and Caiwei Zhong and {El Alam}, Raquelle and Omar Chehab and Joao Lima and Minh Phan and {Dalla Pria}, {Hanna Ferreira} and Alexandra Trevino and Neilan, {Tomas G.} and Kwan, {Jennifer M.} and Vinod Ravi and Hari Deshpande and George Demetri and Choueiri, {Toni K.} and Naqash, {Abdul Rafeh}",

note = "Publisher Copyright: {\textcopyright} 2024 The Authors",

year = "2024",

month = feb,

doi = "10.1016/j.jaccao.2023.11.007",

language = "English (US)",

volume = "6",

pages = "71--79",

journal = "JACC: CardioOncology",

issn = "2666-0873",

publisher = "Elsevier Inc.",

number = "1",

}

TY - JOUR

T1 - Clinical Outcomes Among Immunotherapy-Treated Patients With Primary Cardiac Soft Tissue Sarcomas

T2 - A Multicenter Retrospective Study

AU - Nassar, Amin H.

AU - El-Am, Edward

AU - Denu, Ryan

AU - Abou Alaiwi, Sarah

AU - El Zarif, Talal

AU - Macaron, Walid

AU - Abdel-Wahab, Noha

AU - Desai, Aakash

AU - Smith, Caleb

AU - Parikh, Kaushal

AU - Abbasi, Muhannad

AU - Bou Farhat, Elias

AU - Williams, James M.

AU - Collins, Jeremy D.

AU - Al-Hader, Ahmad

AU - McKay, Rana R.

AU - Malvar, Carmel

AU - Sabra, Mohamad

AU - Zhong, Caiwei

AU - El Alam, Raquelle

AU - Chehab, Omar

AU - Lima, Joao

AU - Phan, Minh

AU - Dalla Pria, Hanna Ferreira

AU - Trevino, Alexandra

AU - Neilan, Tomas G.

AU - Kwan, Jennifer M.

AU - Ravi, Vinod

AU - Deshpande, Hari

AU - Demetri, George

AU - Choueiri, Toni K.

AU - Naqash, Abdul Rafeh

PY - 2024/2

Y1 - 2024/2

N2 - Background: Primary cardiac soft tissue sarcomas (CSTS) affect young adults, with dismal outcomes. Objectives: The aim of this study was to investigate the clinical outcomes of patients with CSTS receiving immune checkpoint inhibitors (ICIs). Methods: A retrospective, multi-institutional cohort study was conducted among patients with CSTS between 2015 and 2022. The patients were treated with ICI-based regimens. The Kaplan-Meier method was used to estimate overall survival (OS) and progression-free survival (PFS). Objective response rates were determined according to Response Evaluation Criteria in Solid Tumors version 1.1. Treatment-related adverse events were graded per the Common Terminology Criteria for Adverse Events version 5.0. Results: Among 24 patients with CSTS, 17 (70.8%) were White, and 13 (54.2%) were male. Eight patients (33.3%) had angiosarcoma. At the time of ICI treatment, 18 patients (75.0%) had metastatic CSTS, and 4 (16.7%) had locally advanced disease. ICIs were administered as the first-line therapy in 6 patients (25.0%) and as the second-line therapy or beyond in 18 patients (75.0%). For the 18 patients with available response data, objective response rate was 11.1% (n = 2 of 18). The median PFS and median OS in advanced and metastatic CSTS (n = 22) were 5.7 months (95% CI: 2.8-13.3 months) and 14.9 months (95% CI: 5.7-23.7 months), respectively. The median PFS and OS were significantly shorter in patients with cardiac angiosarcomas than in those with nonangiosarcoma CSTS: median PFS was 1.7 vs 11 months, respectively (P < 0.0001), and median OS was 3.0 vs 24.0 months, respectively (P = 0.008). Any grade treatment-related adverse events occurred exclusively in the 15 patients with nonangiosarcoma CSTS (n = 7 [46.7%]), of which 6 (40.0%) were grade ≥3. Conclusions: Although ICIs demonstrate modest activity in CSTS, durable benefit was observed in a subset of patients with nonangiosarcoma, albeit with higher toxicity.

AB - Background: Primary cardiac soft tissue sarcomas (CSTS) affect young adults, with dismal outcomes. Objectives: The aim of this study was to investigate the clinical outcomes of patients with CSTS receiving immune checkpoint inhibitors (ICIs). Methods: A retrospective, multi-institutional cohort study was conducted among patients with CSTS between 2015 and 2022. The patients were treated with ICI-based regimens. The Kaplan-Meier method was used to estimate overall survival (OS) and progression-free survival (PFS). Objective response rates were determined according to Response Evaluation Criteria in Solid Tumors version 1.1. Treatment-related adverse events were graded per the Common Terminology Criteria for Adverse Events version 5.0. Results: Among 24 patients with CSTS, 17 (70.8%) were White, and 13 (54.2%) were male. Eight patients (33.3%) had angiosarcoma. At the time of ICI treatment, 18 patients (75.0%) had metastatic CSTS, and 4 (16.7%) had locally advanced disease. ICIs were administered as the first-line therapy in 6 patients (25.0%) and as the second-line therapy or beyond in 18 patients (75.0%). For the 18 patients with available response data, objective response rate was 11.1% (n = 2 of 18). The median PFS and median OS in advanced and metastatic CSTS (n = 22) were 5.7 months (95% CI: 2.8-13.3 months) and 14.9 months (95% CI: 5.7-23.7 months), respectively. The median PFS and OS were significantly shorter in patients with cardiac angiosarcomas than in those with nonangiosarcoma CSTS: median PFS was 1.7 vs 11 months, respectively (P < 0.0001), and median OS was 3.0 vs 24.0 months, respectively (P = 0.008). Any grade treatment-related adverse events occurred exclusively in the 15 patients with nonangiosarcoma CSTS (n = 7 [46.7%]), of which 6 (40.0%) were grade ≥3. Conclusions: Although ICIs demonstrate modest activity in CSTS, durable benefit was observed in a subset of patients with nonangiosarcoma, albeit with higher toxicity.

KW - cardiac sarcomas

KW - cardiac tumors

KW - immune checkpoint inhibitors

KW - treatment-related adverse events

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SN - 2666-0873

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JO - JACC: CardioOncology

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Clinical Outcomes Among Immunotherapy-Treated Patients With Primary Cardiac Soft Tissue Sarcomas: A Multicenter Retrospective Study

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