좌심방의 원발성 미분화성 육종

원발성 미분화 심장육종은 그 빈도가 매우 드물고 공격적인 국소진행을 보인다. 그러므로 수술로 완전절제를 하고, 이후 보조적 항암화학요법 또는 방사선 치료를 하더라도 예후는 아주 불량한 것으로 알려져 있다. 저자들은 33세의 여자 환자에서 원발성 심장종양 중 미분화성육종을 확인한 경우로 수술 후 항암화학요법과 방사선 치료를 하였음에도 불구하고 재발하여 진단 후 11개월만에 사망한 증례 보고와 이에 관한 문헌고찰을 보고하는 바 있다. Primary undifferentiated cardiac sarcomas are extremely rare, and typically undergo aggressive local spread. The prognosis of undifferentiated sarcoma is reported to be very poor even if surgical resection, with or without adjuvant chemotherapy and radiotherapy, is performed. Here we present our case of primary undifferentiated sarcoma of the left atrium. A 33-year-old woman presented with a month history of progressive breathlessness and exertional chest pain. Transthoracic echocardiography showed a large mobile mass obstructing much of the left atrium. At surgery a superseptal approach to the atria enabled removal of all the left heart tumor which was attached to the left upper pulmonary vein. Immunochemistry staining revealed the undifferentiated atrial sarcoma. She received three cycles of darcabazine and adriamycin and after three months three was evidence of tumor recurrence. Eleven months later from operation, she died with congestive heart failure.