Primary sarcoma of pulmonary artery and valve: multimodality treatment by chemotherapy and homograft replacement.
Primary sarcoma of pulmonary artery and valve: multimodality treatment by chemotherapy and homograft replacement.
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原发性肺动脉和瓣膜肉瘤：化疗和同种移植物替代的多模式治疗。
DOI：
10.1016/s0022-5223(96)70120-4
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发表时间：
1996
期刊：
影响因子：
--
通讯作者：
J. Knolle
中科院分区：
文献类型：
--
作者：
H. Zerkowski;H. Hofmann;I. Gybels;J. Knolle
原发性肺肉瘤是极其罕见的肿瘤。自1923年Mandelstamm首次描述以来，仅发表了约100个病例报告，并且只有少数涉及原发性肉瘤的联合治疗。我们报道了一名患有肺动脉瓣转移性肺肉瘤并浸润肺动脉的女性。通过综合化疗使肺转移完全缓解后，患者接受了肿瘤的根治性切除并插入了冷冻保存的同种移植物。一名 49 岁的女性因运动时呼吸急促加剧而入院。她大约 6 个月前患有心包炎。从那时起，胸部和背部疼痛频繁发生，并伴有心动过速和呼吸困难。听诊发现左第二肋间有 3/6 收缩期喷射性杂音和明显分裂的第二心音。心电图有右心室负荷过重的迹象。最初的胸片显示双侧肺部有几个结节性病变，经计算机断层扫描证实。二维和经食管超声心动图显示右心室增大，主肺动脉和右肺动脉结构异常。右心导管检查显示右心室和肺动脉压力升高，心室-远端肺动脉压差为 40 mmHg。通过电影血管造影发现主肺动脉和右肺动脉灌注不完全。对肺部结节性病变进行针吸活检进行细胞学和组织学检查，我们怀疑该病变，后来证实为血管肉瘤。由于危及生命的血流动力学不稳定，右侧肺转移瘤的选择性切除不完整。其中一个肿瘤的组织学检查显示肉芽组织和疤痕组织，没有恶性肿瘤的证据。
Primary pulmonary sarcomas are extremely rare tumors. Since the first description by Mandelstamm 1 in 1923, only about 100 case reports have been published, and only a few have dealt with combined treatment of primary sarcoma. We report on a woman with a metastasizing pulmonary sarcoma of the pulmonary valve with infiltration of the pulmonary artery. After complete remission of the pulmonary metastases with polychemotherapy, the patient underwent curative resection of the tumor and insertion of a cryopreserved homograft.A 49-year-old woman was admitted because of increasing short of breath with exercise. She had pericarditis about 6 months previously. Since then, both chest and back pain occurred frequently, associated with tachycardia and dyspnea. Auscultation revealed a 3/6 systolic ejection murmur in the second left intercostal space and a markedly split second heart sound. There were signs of right ventricular overload on the electrocardiogram. The initial chest radiograph showed several bilateral pulmonary nodular lesions, confirmed by computed tomographic scan. Two-dimensional and transesophageal echocardiography revealed an enlarged right ventricle and an abnormal structure in the main and right pulmonary arteries. Right heart catheterization showed elevated right ventricular and pulmonary arterial pressures, with a ventricular-distal pulmonary artery gradient of 40 mmHg. The main and right pulmonary arteries were seen by cineangiography to be incompletely perfused. Needle biopsy was carried out for cytologic and histologic examination of the pulmonary nodular lesions, which we had suspected and later confirmed to be hemangiosarcoma. Elective resection of the right sided lung metastases was incomplete as a result of life-threatening hemodynamic instability. Histologic examination of one of these tumors revealed granulation and scar tissue without evidence of malignancy.