Primary Intimal Sarcoma of the Left Atrium: An Incidental Finding on Routine Echocardiography

Abstract

Cardiac sarcomas are extremely rare primary malignant tumors of the heart. In this article, we present the case of a 70-year-old female, who was found to have a left atrial mass during a routine outpatient transthoracic echocardiography. Further investigation with cardiac magnetic resonance imaging confirmed the presence of a bilobulated mass with heterogeneous enhancement. Left atrial myxoma was the first diagnostic consideration, followed by other primary cardiac tumors, and thrombus. The patient subsequently underwent resection of the mass, utilizing cardiopulmonary bypass. Upon pathological examination, the mass was found to be an intimal sarcoma. The objective of this report is to describe a case of this rare disease entity, and to discuss its presentation, pathological findings and management.

Functional Mitral Stenosis: Imposture of Primary Cardiac Intimal Sarcoma

“…This is a rare case of primary cardiac intimal sarcoma presenting with functional mitral stenosis and heart failure. Cardiac intimal sarcomas are endocardial mesenchymal tumors that most commonly arise from great vessels like the pulmonary artery or thoracic aorta and are rarely seen in the heart with most originating in the left heart [ 7 , 8 ]. Like in our case, cardiac intimal sarcomas are common in women older than 40 years of age and about 65% of those that arise in the heart originate from the left atrium away from the septal wall unlike most of the myxomas that originate from the inter-atrial septum [ 7 ].…”

“…Other uncommon presentations include atrial fibrillation and other conduction abnormalities, pericardial effusion, signs and symptoms from metastasis; pulmonary metastases (40%) and extrathoracic metastasis (20%) involving kidneys, lymphnodes, brain and skin [ 12 ]. Patients may also be asymptomatic until later stages of the disease [ 8 ]. TTE, TEE, CT scan and cardiac MRI may aid in the diagnostic process; however, definitive diagnosis is established by histopathology [ 11 ].…”

“…The life expectancy is twice as long for patients who undergo complete tumor resection, which indicates that early diagnosis and treatment have important prognostic and therapeutic implications [ 15 ]. Often cardiac intimal sarcomas recur locally and metastasize early in less than 1 year [ 8 ]. Genomic analysis showing frequent alteration in genes like MDM2, PDGFRA and EGFR has led to further research on targeted therapeutic approach [ 7 ], thus, paving a way for other treatment modalities such as chemotherapy and radiation therapy for the treatment of cardiac intimal sarcomas [ 8 ].…”

“…Often cardiac intimal sarcomas recur locally and metastasize early in less than 1 year [ 8 ]. Genomic analysis showing frequent alteration in genes like MDM2, PDGFRA and EGFR has led to further research on targeted therapeutic approach [ 7 ], thus, paving a way for other treatment modalities such as chemotherapy and radiation therapy for the treatment of cardiac intimal sarcomas [ 8 ]. Chemotherapy regimens that may have survival benefit in cardiac intimal sarcoma patients include, ifosfamide-epirubicin (or doxorubicin) and CyVADIC (cyclophosphamide, vincristine, doxorubicin and dacarbazine), which are mainly used for soft tissue sarcomas [ 16 ].…”

“…However, a recent retrospective analysis by Neuville et al, showed cardiac intimal sarcomas (42%) to be the most frequent primary cardiac sarcoma followed by angiosarcoma (26%), undifferentiated sarcoma (22%), synovial sarcomas (7%), leiomyosarcomas (2%) and peripheral neuroectodermal tumor (1%) [ 7 ]. Cardiac intimal sarcomas are encountered more commonly in the large arterial blood vessels including pulmonary artery and aorta, and are extremely rare in the heart [ 4 , 8 - 12 ].…”