Current clinical management of primary cardiac sarcoma

ABSTRACT

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.

KEYWORDS:

• Angiosarcoma
• Heart Cancer
• Cardiac Sarcoma
• Sarcoma

Article highlights

• Primary cardiac tumors are rare, and the vast majority of tumors are benign.

• Primary cardiac sarcomas (PCS) comprise the majority of malignant cardiac tumors and carry an overall poor prognosis of less than 1 year.

• Clinical presentation is dependent on tumor location, with left-sided tumors more likely to present with heart failure and hemodynamic compromise.

• Angiosarcoma is the most common histology and has a worse prognosis.

• Complete surgical resection has the greatest impact on overall survival, however achieving this is challenging given the deep, visceral location of tumors.

• Chemotherapy improves survival and when given neoadjuvantly, may improve the ability to achieve negative margins.

Management of PCS is complex and requires subspecialty, multidisciplinary coordination.

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper received no funding.