Primary Cardiac Sarcomas: A Comprehensive Review of Clinical Characteristics, Diagnosis, Treatment Options, Prognosis, and a Case Report

Primary cardiac sarcomas (PCS) are exceedingly rare malignant tumors originating from the heart. Sarcomas are the predominant malignant primary cardiac neoplasm. They are associated with a poor prognosis due to their aggressive nature, non-specific symptoms leading to delayed diagnosis and limited therapeutic options. Here, we discuss the case of a 45-year-old female who presented with progressive dyspnea and syncope. Imaging revealed a 6.3 × 4.8 × 3.9 cm mass arising from the left ventricle. Biopsy confirmed a malignant spindle cell neoplasm consistent with sarcoma. PET-CT indicated metabolic activity in the left para cardiac region without systemic metastases. Surgical excision was initially planned but deemed high risk; the patient declined surgery and definitive radiation. The patient has been on pazopanib 400 mg twice daily for 6 months, with no major side effects or disease progression. PCS often leads to local tumor progression and systemic metastasis, contributing to high disease–specific mortality. Maximal safe surgical resection offers the best chance for cure in localized disease, although achieving negative margins is challenging. The role of adjuvant therapies such as radiation and chemotherapy remains unclear due to limited survival benefits observed in advanced cases. Neoadjuvant therapy may render borderline inoperable tumors amenable to surgical resection. Emerging therapies like tyrosine kinase inhibitors (TKIs) such as pazopanib show promise in managing advanced disease by targeting specific pathways involved in tumor growth. Further research is needed to establish effective treatment protocols and improves outcomes for patients with this challenging malignancy.