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原発性心臓肉腫はまれな疾患である。標準的な治療は明確ではないが,手術療法による腫瘍切除が生存に寄与するとされる。切除断端陰性の完全切除が望ましいが,肉腫は周囲に浸潤しやすく,また心臓では十分な切除縁確保は困難であり微視的・肉眼的な残存には補助療法として放射線治療が選択されることが多い。また肉腫は遠隔転移をすることも多いため全身化学療法も重要で,生存に寄与するとの報告もある。そのため,手術,化学療法および放射線治療の集学的治療が重要とされる1)2)。
Primary cardiac sarcoma is rare and associated with poor prognosis. Although effective treatment is currently unavailable, reportedly, surgery as well as multimodality therapy including chemotherapy and radiotherapy may improve survival. We describe four patients with cardiac sarcoma;three patients underwent postoperative radiotherapy, and one patient received radiotherapy for postoperative local recurrence.
Among four patients, one survived and was disease-free, whereas three patients died of distant metastases. Two patients underwent resection and radiotherapy and have survived for 9 and 8 months, respectively. The other two patients underwent resection and postoperative radiotherapy and chemotherapy and achieved disease-free survival of 24 months and overall survival of 20 months, respectively. Three patients who received adjuvant therapy within 2 months postoperatively had local tumor control, whereas one patient who did not receive adjuvant therapy developed local recurrence, with consequent respiratory failure. Our results suggest that early postoperative multimodal treatment including radiotherapy prolongs survival and improves local tumor control.
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