The Rarest of the Rare: A Case of Primary Cardiac Osteosarcoma With a Review of the Literature

Abstract

A 54-year-old female presented with shortness of breath and cyanosis. Work up with chest X-ray and subsequent echocardiogram revealed an intracardiac bi-atrial mass leading to emergent cardiothoracic resection. Pathology was consistent with a primary cardiac high-grade osteosarcoma. Post-resection staging positron emission tomography-computed tomography (PET-CT) showed hypermetabolic mixed lytic and sclerotic lesion of T10 concerning for metastasis. She received five cycles of adriamycin and ifosfamide chemotherapy before discontinuation due to systolic dysfunction. Nine months later, she developed a high tumor burden with progressive disease and was treated with second-line gemcitabine/docetaxel with disappointing results. She is currently on treatment with cyclophosphamide and topotecan as third-line treatment with an excellent clinico-radiographic response. Osteosarcomas are aggressive with a high incidence of recurrence and metastasis. Fewer than 50 cases of primary cardiac osteosarcomas have been reported in the literature. Even though complete resection can be achieved in some cases, long-term results are usually poor. No standard therapy has been established.

A Rare Case of Undifferentiated Pleomorphic Cardiac Sarcoma

“…The most commonly used chemotherapeutic regimen for cardiac sarcomas is the combination of ifosfamide and doxorubicin [ 8 ]. Cardiac sarcomas, unfortunately, have a poor prognosis, with an average survival of 6 months to 25 months after diagnosis [ 7 , 13 ].…”

Primary Osteosarcoma of Left Atrial Appendage in a Patient with Rheumatic Heart Disease

“…Primary cardiac osteosarcoma has been reported in about 50 cases over the last 50 years, from the first case described by Dorney in 1967[Dorney 1967Wang 2016;Mhadgut 2021]. Primary cardiac osteosarcoma root in LAA has not been reported.…”