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[一例术前诊断的原发性肺平滑肌肉瘤]

[A case of preoperatively diagnosed primary pulmonary leiomyosarcoma].

作者信息

Amano S, Tanabe N, Yasuda J, Okada O, Yokoi S, Iizasa T, Hiroshima K, Kuriyama T

机构信息

Department of Chest Medicine, Chiba University School of Medicine, Japan.

出版信息

Nihon Kokyuki Gakkai Zasshi. 1999 Dec;37(12):1019-23.

Abstract

A 29-year-old woman had been suffering from right back pain for 3 months. Chronic pulmonary thromboembolism was suspected and she was referred to our hospital. She presented with no risk factors for thromboembolism, and during the previous 6 months had lost 4 kg in body weight. Chest radiography showed nodular shadows in the lower field of the right lung. Contrast-enhanced computed tomography demonstrated a filling defect in the right pulmonary artery and nodular lesions in the lower field of the right lung, which were considered to be signs of pulmonary infarction. Absence of perfusion into the right lung was demonstrated by a perfusion scan. Right heart catheterization showed normal pressure in the pulmonary arteries, and pulmonary angiography showed an abrupt cutoff of the right pulmonary artery, which was similar to the finding of pulmonary thromboembolism. A transvenous catheter suction biopsy was performed in the right pulmonary artery and the histopathologic findings yielded a diagnosis of leiomyosarcoma. The patient underwent surgical resection under total cardiopulmonary bypass. A large tumor completely filled the right main pulmonary artery and invaded the posterior wall of the pulmonary trunk close to the left main pulmonary artery. Primary pulmonary leiomyosarcoma is a rare tumor and its prognosis is very poor. Radical surgical resection is the only effective treatment, but early diagnosis is very difficult. Transvenous catheter suction biopsy is a useful procedure for the early diagnosis of pulmonary artery sarcoma.

摘要

一名29岁女性右背痛3个月。怀疑患有慢性肺血栓栓塞症,遂转诊至我院。她没有血栓栓塞的危险因素,且在过去6个月内体重减轻了4千克。胸部X线检查显示右肺下野有结节状阴影。增强计算机断层扫描显示右肺动脉有充盈缺损,右肺下野有结节状病变,考虑为肺梗死征象。灌注扫描显示右肺无灌注。右心导管检查显示肺动脉压力正常,肺血管造影显示右肺动脉突然中断,这与肺血栓栓塞的表现相似。在右肺动脉进行了经静脉导管抽吸活检,组织病理学检查结果诊断为平滑肌肉瘤。患者在全心肺转流下接受了手术切除。一个大肿瘤完全充满了右主肺动脉,并侵犯了靠近左主肺动脉的肺动脉干后壁。原发性肺平滑肌肉瘤是一种罕见肿瘤,预后很差。根治性手术切除是唯一有效的治疗方法,但早期诊断非常困难。经静脉导管抽吸活检是肺动脉肉瘤早期诊断的一种有用方法。

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