Key result
Surgical resection of a massive primary pulmonary artery sarcoma was successfully performed, but the patient died of cerebral hemorrhage during postoperative chemotherapy six months later.
Case Report (n=1)
No
Surgical resection using deep hypothermic circulatory arrest can successfully normalize pulmonary artery pressure in patients with primary pulmonary artery sarcoma, though overall prognosis remains challenging.
Rapidly progressive CTEPH-like presentation warrants sarcoma consideration; extends limited surgical case data while leaving optimal management open.
62歳女性．労作時の息切れが急速増悪し，心エコーでTRPG 75 mmHgであることから慢性血栓塞栓性肺高血圧症（CTEPH）を疑われた．肺動脈造影とCTで肺動脈の中枢から末梢にかけて広範囲の可動性のある腫瘤像を認め，急速に悪化した経過と形態，およびFDG-PETで異常集積がみられたことから肺動脈原発腫瘍が疑われた．予後は不良であると予測されたが，突然死の可能性があり，肺や他の臓器への転移は認められず，耐術可能と思われたので，術後早期に化学療法を行うこととして，腫瘍切除術を適応した．CTEPHに準じ，低体温循環停止法を併用して肺動脈内に充満した腫瘍を摘出し，原発部位と思われた肺動脈弁も切除した後，肺動脈弁置換術と肺動脈修復術を施行した．病理診断は肺動脈原発肉腫（High grade sarcoma）であった．術後，肺動脈圧は正常化し，化学療法を施行されたが，6カ月後に被殻出血で死亡した．稀な肺動脈原発肉腫の1例を文献的考察を加えて報告する．
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Nishiyama et al. (2020) conducted a case report in Primary pulmonary arterial sarcoma with pulmonary hypertension (n=1). Surgical resection and postoperative chemotherapy was evaluated on Clinical outcome. Surgical resection of a massive primary pulmonary artery sarcoma was successfully performed, but the patient died of cerebral hemorrhage during postoperative chemotherapy six months later.
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