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Year : 2019  |  Volume : 56  |  Issue : 1  |  Page : 88-

Cardiac sarcomas: A gist

Pradeep Vaideeswar 
 Department of Pathology (Cardiovascular and Thoracic Division), Seth GS Medical College, Mumbai, Maharashtra, India

Correspondence Address:
Pradeep Vaideeswar
Department of Pathology (Cardiovascular and Thoracic Division), Seth GS Medical College, Mumbai

How to cite this article:
Vaideeswar P. Cardiac sarcomas: A gist.Indian J Cancer 2019;56:88-88

How to cite this URL:
Vaideeswar P. Cardiac sarcomas: A gist. Indian J Cancer [serial online] 2019 [cited 2021 Jan 16 ];56:88-88
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Although heart was considered “an organ too noble to be attacked by a neoplasm,”[1] primary and secondary tumors do occur, of which the latter are about 30 times more common than the former. Nearly 75% of the primary cardiac tumors are benign, while the remaining tumors are constituted by malignant tumors. This issue carries a case report of a primary osteosarcoma,[2] which is a rare subtype of cardiac sarcomas (CS). These sarcomas constitute nearly 75% of primary malignant cardiac tumors, unfortunately affecting young patients (median age of 39–44 years) with no predisposing factors; there is usually no sex predilection.[3] The first sarcoma to be reported was a rhabdomyosarcoma.[4]

The clinical presentation of CS in general depends on a large extent on the site and size of the tumor.[3] The symptoms may be constitutional or related to obstruction, local infiltrative growth, systemic embolization, or metastases.[3] Some can, however, remain clinically silent until they reach a large size. The histopathology is no different from extracardiac soft tissue sarcomas, and all types exist. However, there are important characteristics.[5] Angiosarcomas, leiomyosarcomas, and undifferentiated pleomorphic sarcomas are the most common subtypes. It has been noted that right-sided sarcomas tend to be more bulky and infiltrative, with increased metastatic potential. Most sarcomas are located in the right atrium and are angiosarcomas. On the other hand, the left-sided sarcomas are often seen in the left atrium forming polypoidal masses, simulating the more common benign cardiac myxomas and frequently exhibit the histomorphology of undifferentiated sarcomas. It is of no surprise, therefore, that even primary cardiac osteosarcomas preferentially arise in the left atrium, as seen in the case report.

Primary cardiac osteosarcoma is an uncommon tumor, forming less than 10% of the CS; recently there has been an elaborate review of 53 patients with these tumors.[6] The osteosarcoma arises from undifferentiated mesenchymal stem cells capable of divergent differentiation, which would explain the debate to classify it as undifferentiated sarcoma with osteosarcomatous differentiation,[6] and also the smooth muscle actin positivity seen in the reported case.

Imaging modalities such as echocardiography, computed tomography, magnetic resonance imaging, and positron emission tomography are important to not only delineate the cardiac tumors, but also to assess the degree of cardiac impairment, metastatic disease, and very importantly to rule out a soft tissue/bone primary tumor.[3] Complete or near-complete tumor resection, chemoradiotherapy, autotransplantation, and transplantation are the multiple treatment options required, but they usually are associated with good early clinical outcome.[7]


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