Left atrial myxosarcoma with systemic metastasis: A case report

The term myxosarcoma is currently not used in standard classification for s oft tissue tumors, but restricted to cardiac tumors. Primary cardiac myxosa rcoma is a very rare disease and is difficult to differentiate from myxoma clinically and pathologically. We report a case of left atrial myxosarcoma with widespread systemic metastasis in a 21-yr-old male. The patient presen ted with sudden onset of intermittent dyspnea and orthopnea. Echocardiograp hy showed a mobile, pedunculated tumor, 7.5 x 5 x 2 cm in size, at left atr ium. Histologically, the excised tumor showed an amorphous finely fibrillar and mucinous stroma, in which irregular cords and clusters of lepidic cell s and large stellate cells with plump vesicular nuclei resembled the usual type of cardiac myxoma were noted. And it showed focally cellular area with great nuclear pleomorphism and frequent mitoses. The patient received comb ination chemotherapy, peripheral blood stem cell collection transplantation and operations for systemic metastases in the brain, skeletal muscle and l ung. He is alive at present 37 months after initial diagnosis and has no mo re new metastatic lesion.