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[原发性肺滑膜肉瘤——一种罕见肿瘤]

[Primary synovial sarcoma of the lung - a rare tumor].

作者信息

Krieghoff B, Skuballa A, Leonhardt P, Mohr F-W, Wittekind C, Bossert T, Achatzy R

机构信息

Städt. Klinikum "St. Georg", Klinik für Thorax- und Gefässchirurgie, Robert Koch-Klinik, Germany.

出版信息

Zentralbl Chir. 2002 Aug;127(8):716-9. doi: 10.1055/s-2002-33580.

Abstract

We present a 26 year old patient with a primary malignant synovial sarcoma of the lung that was observed for more than one year by a general practitioner and a pulmologist. Finally, because of recurrent hemoptysis a central tumor of mesenchymal origin of the left lower lobe was diagnosed by bronchoscopy. The invasion of the left atrium as far as to the mitral valve was diagnosed by MRT. The patient was operated on by extended pneumonectomy with extracorporeal circulation. The partial excision of the left atrium required plastic reconstruction. In the postoperative course the patient underwent chemo-therapy, 6 cycles adriablastine/ifosfamid. 8 months after the first operation an extensive tumor recurrency occurred with infiltration of the chest wall. The patient refused further radio- or chemotherapy and died 14 months after the operation. Because of the small number of cases therapeutic strategy conceptions do not exist. The resection of the tumor is generally recommended. Chemo- and radiotherapy are accepted as an option for advanced tumor stage.

摘要

我们报告一名26岁的患者,患有原发性肺恶性滑膜肉瘤,由一名全科医生和一名肺科医生观察了一年多。最后,由于反复咯血,通过支气管镜检查诊断为左肺下叶间叶源性中央肿瘤。磁共振成像(MRT)诊断出肿瘤侵犯左心房直至二尖瓣。患者接受了体外循环下的扩大肺切除术。左心房部分切除需要整形重建。术后患者接受了化疗,共6个周期的阿霉素/异环磷酰胺。首次手术后8个月出现广泛的肿瘤复发并侵犯胸壁。患者拒绝进一步的放疗或化疗,术后14个月死亡。由于病例数量少,不存在治疗策略的概念。一般建议切除肿瘤。化疗和放疗被认为是晚期肿瘤阶段的一种选择。

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