Combined treatment for a large primary cardiac sarcoma: a case report

BACKGROUND: Undifferentiated pleomorphic sarcoma (UPS) as a primary cardiac tumor is rare, with extremely poor prognosis owing to high recurrence and invasion. We encountered a patient who presented with a primary cardiac tumor incarcerating the mitral valve and who was in a shock state. CASE PRESENTATION: A 41-year-old man was transported emergently to our hospital owing to acute respiratory distress and hemoptysis. He was diagnosed with acute left heart failure caused by a large mass in the left atrium (LA) that obstructed cardiac blood flow, as revealed by imaging study findings, and he underwent an emergency open-heart surgery for tumor resection. He was pathologically diagnosed with UPS invading the muscle layer of the LA. However, after receiving combined therapy for local recurrence and distant metastasis, including proton beam radiotherapy and chemotherapy with molecularly targeted drugs, he could return to work for 2 more years after surgery. CONCLUSION: In this study, we reported the case of a patient who was in a state of shock state owing to the presence of UPS in the LA. The patient underwent an emergency surgery and received combined therapy. He survived for 2 more years after an initial diagnosis, without active local recurrence and distant metastasis.