Primary Cardiac Sarcoma in Pregnancy: A Case Report

Abstract

Primary cardiac sarcoma is a rare disease in adults. It is also associated with poor prognoses, due to diagnostic delay, therapeutic difficulty, and high metastatic potential. The coincidence of pregnancy and a primary cardiac intimal sarcoma is extremely rare. We report a pregnant woman at 27+5 weeks gestation who was admitted to the hospital with acute-onset dyspnea. A mass was found on the left atrium by transthoracic echocardiography. Subsequently, the intracardiac mass was removed, and mitral valve replacement and modified DeVega tricuspid annuloplasty were performed. The patient was diagnosed with a undifferentiated sarcoma, and gave birth to a 1,230 g living baby boy by Caesarean section from preterm contraction at 29+5 weeks gestation. The patient then received systemic chemotherapy. However, 10 months after the initial clinical onset, the patient suddenly died. Surgery is the standard treatment for cardiac tumors, and their removal should always be attempted, even in pregnant women. Although the overall survival rates of the patients are rather poor, palliative cardiac surgery allows the prolonging of pregnancy, until an acceptable fetal viability level is reached.

Role of Multimodal Cardiac Imaging in Diagnosing a Primary Intimal Sarcoma of the Left Atrial Appendage

“…[4][5][6] However, intimal sarcomas that arise from the left atrium, as in our patient, have been reported infrequently. [7][8][9][10][11][12] Our patient's case illustrates the value of multimodal imaging in evaluating cardiac masses. The symptoms caused by cardiac tumors depend on their size, location, and degree of outf low obstruction; they often include dyspnea, chest discomfort, pericardial effusion, tamponade, and heart failure.…”

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

“…Symptomatic cardiac sarcomas usually present with dyspnea, orthopnea, paroxysmal nocturnal dyspnea, or lower extremity edema. 5,8 Patients can also present with the features of metastatic disease in advanced stages. 7 …”

Right atrial spindle cell sarcoma as a rare cause of tricuspid stenosis

“…They lack a specific histology, with negative immunohistochemistry for desmin, factor VIII and keratin. They are extremely rare in heart, with only few cases reported so far 9 10. Prognosis depends on the grade of the tumour, which is based on the mitotic activity, necrosis and degree of cellular differentiation.…”