The endometrial stromal sarcoma is a rare neoplasm of the uterine origin. Intracardiac metastasis form the low-grade endometrial stromal sarcoma is an extremely rare event. A case of a patient who underwent surgical resection of metastatic tumors of the low-grade endometrial stromal sarcoma in the right atrium is described in the present report. Cardiac malignancies account for less than 1% of cardiac surgery and around 0.1% of cardiac echo graphic studies. The presence of metastatic tumor to the heart usually indicates widespread metastases. Lung carcinomas are the most commonly encountered tumor followed by breast and pancreas cancer and melanoma.We show the case report of a woman with endometrial stromal sarcoma who developed a right heart failure, lower extremity edema, dyspnea and orthopnea after the initial step of comprehensive surgery and radiotherapy. Heart metastases was diagnosed by computed tomography and echocardiophy after a palliative metastasectomy; the patient received chemotherapy and hormotherapy without showing response, re-developing a heart metastasic tumor. Tumoral activity was the cause of her death.It is very difficult to standardize care procedures for these patients due to the rarity of this condition. However, it is possible that aggressive therapy (surgery plus radiotherapy and chemotherapy) may lengthen patients survival and improve life quality.
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