{Reference Type}: Journal Article
{Title}: Primary cardiac sarcoma: demographics, genomic study correlation, and survival benefits of surgery with adjuvant therapy in U.S. population.
{Author}: Khan R;Sunthankar KI;Yasinzai AQK;Tareen B;Zarak MS;Khan J;Nasir H;Nakasaki M;Jahangir E;Heneidi S;Ullah A;
{Journal}: Clin Res Cardiol
{Volume}: 113
{Issue}: 5
{Year}: 2024 May 29
{Factor}: 6.138
{DOI}: 10.1007/s00392-023-02236-x
{Abstract}: <B>BACKGROUND: </B>Cardiac sarcomas are rare and aggressive tumors with little known about the demographics, genetics, or treatment outcomes.<BR><B>OBJECTIVE: </B>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.<BR><B>METHODS: </B>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.<BR><B>RESULTS: </B>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.<BR><B>CONCLUSIONS: </B>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.