Bleisch V R, Kraus F T
Cancer. 1980 Jul 15;46(2):314-24. doi: 10.1002/1097-0142(19800715)46:2<314::aid-cncr2820460217>3.0.co;2-t.
A case of polypoid sarcoma of the pulmonary trunk is described in an 80-year-old woman who had a 21-year history of episodic chest pain and hemoptysis. Ultrastructural examination revealed Z bands, characteristic of rhabdomyosarcoma, and leptomeric organelles. This is the 60th reported case of sarcoma of the pulmonary trunk. Symptoms in reported cases were variable, usually caused by tumor emboli to the lungs or by right ventricular outflow obstruction. Angiography was diagnostic. The tumor was characteristically polypoid, often multicentric, and by definition was fixed to the pulmonary trunk or valves. Non-differentiated sarcoma (often uniquely pleomorphic) was found in 37% of patients, leiomyosarcoma (once confirmed by electron microscopy) in 17%, myxosarcoma in 13%, elements of rhabdomyosarcoma in 8%, fibrosarcoma in 8%, elements of chondrosarcoma in 11%, and 5% were malignant mesenchymomas. The authors suggest that these tumors originate from the undifferentiated tissues of the bulbus cordis and propose the name myenchymoma for the tumor.
本文描述了一例发生在一位80岁女性的肺动脉干息肉样肉瘤病例，该患者有21年发作性胸痛和咯血病史。超微结构检查发现了横纹肌肉瘤特有的Z带和细肌丝细胞器。这是第60例报道的肺动脉干肉瘤病例。报道病例中的症状各不相同，通常由肺部肿瘤栓塞或右心室流出道梗阻引起。血管造影具有诊断价值。该肿瘤的特征是息肉样，常为多中心性，根据定义固定于肺动脉干或瓣膜。37%的患者为未分化肉瘤（通常具有独特的多形性），17%为平滑肌肉瘤（经电子显微镜确诊），13%为黏液肉瘤，8%为横纹肌肉瘤成分，8%为纤维肉瘤，11%为软骨肉瘤成分，5%为恶性间叶瘤。作者认为这些肿瘤起源于动脉圆锥的未分化组织，并为该肿瘤提出了间叶瘤这一名称。
