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Primary cardiac sarcoma: demographics, genomic study correlation, and survival benefits of surgery with adjuvant therapy in U.S. population

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Clinical Research in Cardiology Aims and scope Submit manuscript

Abstract

Background

Cardiac sarcomas are rare and aggressive tumors with little known about the demographics, genetics, or treatment outcomes.

Objectives

The objectives of this study were to characterize the demographics, treatment modality, and survival associated with cardiac sarcomas and evaluate the potential for mutation-directed therapies.

Methods

All cases from 2000 to 2018 of cardiac sarcoma were extracted from the SEER database. Genomic comparison utilized The Cancer Genome Atlas (TCGA) database, as well as reviews and re-analysis of past applicable genomic studies.

Results

Cardiac sarcomas occurred most often in White patients, compared with national census data cardiac sarcomas occurred at a significantly higher rate in Asians. The majority of cases were undifferentiated (61.7%) and without distant metastases (71%). Surgery was the most common primary treatment modality and offered survival benefit (HR 0.391 (p = 0.001) that was most pronounced and sustained as compared to patients who received chemotherapy (HR 0.423 (p = 0.001) or radiation (HR 0.826 (p = 0.241) monotherapy. There was no difference in survival when stratified by race or sex; however, younger patients (< 50) had better survival. Genomics data on histologically undifferentiated cardiac sarcomas revealed a significant number were likely poorly differentiated pulmonary intimal sarcomas and angiosarcomas.

Conclusions

Cardiac sarcoma is a rare disease with surgery continuing to be a cornerstone of therapy followed by traditional chemotherapy. Case studies have indicated the potential for therapies directed to specific genetic aberrations to improve survival for these patients and utilization of next-generation sequencing (NGS) will help improve both classification and these therapies for cardiac sarcoma patients.

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Data availability

All the data is publicly available both from SEER and TCGA.

Abbreviations

NGS:

Next-generation sequencing

SEER:

Surveillance, epidemiology, and end results

SAS:

Statistical analysis system

PMCT:

Primary malignant cardiac tumors

NCDB:

National Cancer Database

NCCN:

National Comprehensive Cancer Network

NRTK:

Neurotrophic receptor tyrosine kinase

TCGA:

The Cancer Genome Atlas

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Funding

This work did not receive any direct funding resources.

Author information

Author notes

  1. Rozi Khan and Kathryn I. Sunthankar equally contributed and shared the first coauthor.

Authors and Affiliations

  1. Department of Medicine, Medical University of South Carolina, Florence, SC, USA

    Rozi Khan

  2. Division of Cardiology, Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, USA

    Kathryn I. Sunthankar & Eiman Jahangir

  3. Bolan Medical College, Quetta, Pakistan

    Abdul Qahar Khan Yasinzai & Bisma Tareen

  4. Internal Medicine, Howard University Medical Center, Washington, DC, USA

    Muhammad Samsoor Zarak

  5. Department of Pathology, Indiana University School of Medicine, Indianapolis, IN, USA

    Jaffar Khan

  6. St. George’s University, School of Medicine, University Centre Grenada, West Indies, Grenada

    Hassan Nasir

  7. Department of Pathology and Laboratory Medicine, Cedars-Sinai Medical Center, Los Angeles, CA, USA

    Manando Nakasaki & Saleh Heneidi

  8. Department of Pathology, Vanderbilt University Medical Center, Nashville, TN, USA

    Asad Ullah

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Contributions

AU and RK were involved in conceptualization, study design, data collection, figures, data interpretation, and writing—original draft, review, and editing. KIS, AQKY, BT, MSZ, and JK were involved in data collection, figures, data interpretation, and writing—original draft, review, and editing. AU, KIS, AQKY, and SH were involved in supervision, data interpretation, and writing—review and editing. KIS, EJ, SH, and AU were involved in conceptualization, study design, supervision, data interpretation, and writing—review and editing.

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Khan, R., Sunthankar, K.I., Yasinzai, A.Q.K. et al. Primary cardiac sarcoma: demographics, genomic study correlation, and survival benefits of surgery with adjuvant therapy in U.S. population. Clin Res Cardiol 113, 694–705 (2024). https://doi.org/10.1007/s00392-023-02236-x

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