Nonomura A, Kurumaya H, Kono N, Nakanuma Y, Ohta G, Terahata S, Matsubara F, Matsuda T, Asaka T, Nishino T
Department of Pathology, School of Medicine, Kanazawa University Hospital, Kanazawa University, Japan.
Acta Pathol Jpn. 1988 Jul;38(7):883-96. doi: 10.1111/j.1440-1827.1988.tb02360.x.
Two cases of primary pulmonary artery sarcoma are reported. The patient in the first case was a 61-year-old male with a two-year history of cough and exertional dyspnea, who died of intractable cardiac failure two months after admission without establishment of a diagnosis related to the etiology of cardiac failure. Autopsy revealed a sessile tumor within the pulmonary trunk and a solitary metastatic lesion in the lung. Histologic, immunohistochemical and electron microscopic studies were performed and a diagnosis of malignant mesenchymoma was made. The patient in the second case was a 32-year-old male complaining of exertional dyspnea and back pain. Radiologic studies indicated a mediastinal tumor involving the pulmonary artery. Exploratory thoracotomy revealed that the mediastinal mass arose from the left pulmonary artery. He died of respiratory failure 26 months after onset of his initial symptoms. Histologic, immunocytochemical and electron microscopic studies of both surgical and autopsy materials revealed a malignant fibrous histiocytoma. One hundred ten previously reported cases of this tumor are reviewed, and its clinicopathologic and morphologic features and probable histogenesis are discussed.
报告了两例原发性肺动脉肉瘤。第一例患者为61岁男性，有两年咳嗽和劳力性呼吸困难病史，入院两个月后死于顽固性心力衰竭，未能明确心力衰竭的病因诊断。尸检发现肺动脉主干内有一蒂状肿瘤，肺内有一个孤立的转移灶。进行了组织学、免疫组织化学和电子显微镜检查，诊断为恶性间叶瘤。第二例患者为32岁男性，主诉劳力性呼吸困难和背痛。影像学检查显示纵隔肿瘤累及肺动脉。开胸探查发现纵隔肿块起源于左肺动脉。他在出现初始症状26个月后死于呼吸衰竭。对手术和尸检材料进行的组织学、免疫细胞化学和电子显微镜检查显示为恶性纤维组织细胞瘤。回顾了之前报道的110例该肿瘤病例，并讨论了其临床病理和形态学特征以及可能的组织发生。
