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主肺动脉肉瘤:复发性肺栓塞的一种罕见病因。

Sarcoma of the main pulmonary artery: an unusual etiology for recurrent pulmonary emboli.

作者信息

Pagni S, Passik C S, Riordan C, D'Agostino R S

机构信息

Division of Thoracic Surgery, Hospital of Saint Raphael, New Haven, Connecticut 06511, USA.

出版信息

J Cardiovasc Surg (Torino). 1999 Jun;40(3):457-61.

Abstract

We describe a case of primary pulmonary artery (PA) trunk spindle cell sarcoma in an 86 year old female presenting clinically with debilitating signs of recurrent pulmonary embolism. Further extensive work aroused suspicion for pulmonary artery malignancy. Palliative wide surgical resection, pulmonary artery tumor embolectomy and reconstruction of the proximal pulmonary artery and right ventricle outflow tract (RVOT) with bovine pericardial tissue were performed. She survived the procedure with an improved quality of life, but expired due to recurrence at 6 months postoperatively. Albeit uncommon, pulmonary artery sarcoma is nowadays a more frequently preoperatively diagnosed and surgically treated malignancy. With a modern low perioperative mortality, aggressive surgical resection remains as the single most effective modality for its treatment and can result in short term palliation in selected patients.

摘要

我们描述了一例86岁女性原发性肺动脉主干梭形细胞肉瘤病例,临床上表现为复发性肺栓塞的衰弱症状。进一步的广泛检查引发了对肺动脉恶性肿瘤的怀疑。进行了姑息性广泛手术切除、肺动脉肿瘤切除术,并使用牛心包组织重建近端肺动脉和右心室流出道(RVOT)。她术后生活质量有所改善,但术后6个月因复发死亡。尽管肺动脉肉瘤并不常见,但如今它是一种术前诊断和手术治疗更为频繁的恶性肿瘤。鉴于现代围手术期死亡率较低,积极的手术切除仍然是其治疗的最有效单一方式,并且可以使部分患者获得短期缓解。

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