Primary Cardiac Osteosarcoma: Epidemiology, Diagnosis, and Management - A Narrative Review.

Primary cardiac sarcomas are exceedingly rare, with a very low incidence in the general population. A variety of sarcoma subtypes can originate in the heart, including angiosarcoma, rhabdomyosarcoma, leiomyosarcoma, fibrosarcoma, and osteosarcoma. These tumors are typically classified based on histological characteristics, most often determined following surgical excision or biopsy. Among them, primary cardiac osteosarcoma is one of the rarest forms, with fewer than 60 cases reported in the literature to date. This narrative review aims to provide an overview of primary cardiac osteosarcoma, including its epidemiology, clinical presentation, and current management strategies. Additionally, we highlight several remarkable case reports to illustrate the variability and complexity of this rare malignancy. # Summary The clinical presentation of primary cardiac osteosarcoma often mimics other cardiac conditions such as heart failure, mitral stenosis, or coronary artery disease, making diagnosis challenging. Transthoracic echocardiography (TTE) is typically the first imaging modality to detect a cardiac mass. Due to the rarity of the tumor, established treatment protocols are limited. Surgical resection is generally the preferred initial approach for localized disease without metastasis. Chemotherapy and radiation therapy may also be used, but their application must be carefully considered given the heart's sensitivity to these treatments. Despite advances in imaging, surgical techniques, and adjuvant therapies that have improved survival in some cases, the overall prognosis for primary cardiac osteosarcoma remains poor. # Conclusion Because primary cardiac osteosarcoma is so rare, establishing standardized treatment guidelines remains challenging. By reviewing historical cases and examining current clinical management strategies, we aim to contribute to a better understanding of this aggressive malignancy and ultimately improve patient outcomes.