Symptomatic intracavitary (noninvasive) cardiac metastasis from low grade endometrial stromal sarcoma of the uterus.

We report a 49-year-old woman who was operated upon 33 years ago for uterine endometrial stromal sarcoma of low-grade malignancy. The patient showed obstruction of the right ventricular outflow tract. An echocardiogram and MRI showed that a mass emanating from the inferior vena cava filled the right atrium, the right ventricle, and part of the pulmonary artery. The entire intracavitary neoplasm was successfully removed and diagnosed as low-grade endometrial stromal sarcoma. To our knowledge, this is the second report of a low-grade endometrial stromal sarcoma reaching the heart via the inferior vena cava. In this case an intracavitary metastasis may be a feature indicating the progression of the endometrial stromal sarcoma. Therefore, this neoplasm should be included in the differential diagnosis of cardiac intracavitary neoplasms. Patients affected by this tumor may benefit from early identification and resection of the obstructing neoplasm.