Demirci Nilgün Yilmaz, Naurzvai Nurgül, Kirbaş Ismail, Akyürek Nalan, Gürsel Gül, Öztürk Can
Department of Chest Disease, Faculty of Medicine, Gazi University, Ankara, Turkey.
Department of Radiology, Lokman Hekim Hospital, Ankara, Turkey.
Lung India. 2018 Mar-Apr;35(2):164-167. doi: 10.4103/lungindia.lungindia_137_17.
Leiomyosarcomas are rare neoplasms of the smooth muscles. Primary pulmonary leiomyosarcomas, which constitute approximately 0.2%-0.5% of all primary lung malignancies, are extremely rare and highly lethal. They may originate from the smooth muscle cells of the bronchial wall, the blood vessels, or the pulmonary interstitium, and their rare occurrence, localization, and nonspecific clinical symptoms mean that correct diagnosis and proper management are often delayed. Here, we report a rapidly growing primary pulmonary leiomyosarcoma, which invaded the right atrium, vena cava superior, mediastinum, right hilar area, and left pulmonary artery within 4 months. On histopathology, a transthoracic needle biopsy of the mass confirmed leiomyosarcoma, and delayed presentation meant that there was a local spread to the neighboring structures at the time of diagnosis.
平滑肌肉瘤是一种罕见的平滑肌肿瘤。原发性肺平滑肌肉瘤约占所有原发性肺恶性肿瘤的0.2%-0.5%，极为罕见且具有高度致死性。它们可能起源于支气管壁、血管或肺间质的平滑肌细胞，其罕见的发生率、定位以及非特异性临床症状意味着正确诊断和恰当治疗常常被延误。在此，我们报告一例生长迅速的原发性肺平滑肌肉瘤，该肿瘤在4个月内侵犯了右心房、上腔静脉、纵隔、右肺门区及左肺动脉。组织病理学检查显示，经胸壁肿块穿刺活检确诊为平滑肌肉瘤，而就诊延迟意味着在诊断时肿瘤已局部扩散至邻近结构。
