Primary Cardiac Sarcoma Presenting As Acute Left Heart Failure

Case Presentation:

A 35 year old male with no known past medical history presented for the evaluation of his worsening shortness of breath cough with hemoptysis, on and off fever and weight loss of about 20 pounds in the past 2 months. He was a known smoker, and denied any recent travel or contact with tuberculosis patients. Except mild pallor, trace pedal edema and few crepitations at the right lung base, examinations including cardiovascular were unremarkable.

Connective tissue diseases, tuberculosis, lung malignancy, granulomatosis with polyangitis (wegener’s) were ruled out by negative ANA, quantiferone, CT and bronchoscopy, and ANCA respectively.Transthoracic echocardiogram revealed a large left atrial mass 8.15 cm x 2.2 cm attached to interatrial septum. Transesophageal echocardiogram confirmed the above finding,along with the mass protruding across mitral valve into left ventricle with a mild mitral regurgitation, with an invasion to the pulmonary vein and also elevated right ventricular systolic pressure (RVSP) of 120 mmHg with a severe tricuspid regurgitation.

Cardiothoracic surgery was consulted, and only a limited excision of the mass could be done along with mitral valve repair. Histopathology along with immunohistochemical staining of the mass revealed high grade undifferentiated pleomorphic sarcoma. A follow up PET scan showed residual disease activity in the pulmonary vein. Patient was started on chemotherapy with ifosfamide and doxorubicin and was referred to a higher center for further management. Patient has received four cycles of chemotherapy so far and one dose of radiation treatment. At nine months follow up, the patient is doing well without any complications.

Discussion:

Primary cardiac sarcomas (PCS) are rare malignant tumors of the heart. Clinical features depend on the site of tumor and vary from symptoms of congestive heart failure to thromboembolism and arrhythmias. About a third of these tumors have evidence of systemic metastasis at diagnosis. PCS located in the left atrium can present with symptoms of mitral stenosis, as in our case. Our case also had constitutional symptoms such as fever, weight loss and fatigue, which have been reported particularly in atrial myxoma.

Echocardiography is helpful but definitive diagnosis is established by histopathology. A transesophageal echocardiography is superior to a transthoracic echocardiography in identifying the presence and site of origin of primary cardiac tumors. Presence of non septal origin, broad attachment on the wall, extension into pulmonary vein, semisolid consistency and multiple masses can predict the likelihood of a malignant tumor. A chest CT and MRI can be helpful in the better delineation of anatomy for surgical planning as well as for evaluation of tumor extension.

Surgical resection is the mainstay of treatment and the role of chemotherapy and radiotherapy is unclear. The most important predictor of prognosis is the ability to achieve a complete surgical resection of the tumor. The role of neoadjuvant chemotherapy in these cases is unclear. Orthotropic cardiac transplantation has been tried but the experience is limited. Chemotherapy, most commonly with doxorubicin and/or ifosfamide containing regimens has been tried with unclear survival benefit.

Conclusions:

Primary cardiac sarcomas are rare malignant tumors with poor prognosis. Surgery is the treatment of choice, and the role of chemotherapy and radiotherapy is unclear.