Current clinical management of primary cardiac sarcoma

Introduction: Primary cardiac tumors are exceedingly rare, with approximately 75% representing benign lesions. Sarcoma represents the most common primary cardiac malignancy, with a wide range of sarcoma histologies represented. Symptoms at diagnosis vary based on tumor location. Multidisciplinary treatment including chemotherapy, surgery, and occasionally radiation is often warranted. Despite aggressive treatment, the overall prognosis for primary cardiac sarcoma (PCS) remains poor with a median survival of approximately 1 year.Areas covered: A PubMed search for the key terms; 'cardiac sarcoma', 'primary cardiac sarcoma', and 'treatment' were conducted. Abstracts were reviewed for reports on presentation, treatments, and outcomes in PCS. Available data was limited to single-institution series, most of which were retrospective. Patterns of symptoms at diagnosis varied with tumor location (right vs. left vs. pericardium). Multimodality therapy, including chemotherapy and surgical resection was most commonly reported. Completely negative margin (R0) resection has the greatest impact on overall survival.Expert opinion: Given the rarity of PCS, patients should be referred to a high-volume sarcoma center for multidisciplinary evaluation. Neoadjuvant chemotherapy should be considered to aid in surgical resection. Due to the propensity for brain metastases in cardiac tumors, brain MRI at the time of diagnosis should be considered.