摘要目的:分析肺恶性血管周上皮样细胞肿瘤（PEComa）合并腺癌的发病机制、临床表现、诊断、治疗及预后。方法:分析2020年8月南方医科大学附属东莞人民医院病理科确诊的1例肺原发恶性PEComa合并腺癌病例，整理患者的临床资料、病理诊断要点、治疗方案及预后等，并进行文献复习。首先以“恶性血管周上皮样细胞肿瘤”+“肺”+“腺癌”检索中国知网及万方医学数据库，未检索到相关报道。随后以“肺恶性血管周上皮样细胞肿瘤”为检索词检索中国知网及万方医学数据库，同时以“pulmonary malignant perivascular epithelioid cell tumor”和“PEComa”为副主题词，结合主题词，检索PubMed、Embase、Web of Science及Cochrane数据库，检索语言为中文或英文，起始时间不限，检索时间截止日期为2020年11月。结果:患者男，46岁，因“反复咳嗽、胸痛10余天伴体重迅速减低”于2020年8月20日入院。血清学肿瘤标志物示非小细胞肺癌相关抗原表达增高。正电子发射计算机体层扫描术（PET）-CT示左胸腔较大团块状软组织密度灶，SUV值22.8。术后病理诊断为恶性PEComa合并肺腺癌伴淋巴结转移，且检测到驱动基因EGFR药物敏感性突变，术后辅以化疗及靶向治疗，目前状态稳定。文献共检索到肺恶性PEComa 12例，中老年常见，多因咳嗽或胸闷就诊，胸部CT常显示边界清楚的圆形肿块，8例发生纵隔淋巴结及其他器官转移。结论:肺恶性PEComa少见，同一肿物合并肺原发腺癌成分者为首次报道。部分病例进展迅速，手术完整切除病灶并进行淋巴结清扫，术后辅以化疗及靶向治疗，是较为合适的治疗策略。诊断为良性PEComa的患者也应注意长期随诊复查。

abstractsObjective:To analyze the pathogenesis, clinical manifestations, diagnosis, treatment and prognosis of pulmonary malignant perivascular epithelioid cell tumor (PEComa) with adenocarcinoma.Methods:In August 2020, the Department of Pathology, Dongguan People′s Hospital of Southern Medical University, diagnosed a case of pulmonary malignant PEComa mixed with adenocarcinoma. The clinical data, pathological diagnosis, treatment plan and prognosis of the patient were analyzed, and the literature was reviewed. Firstly, "malignant perivascular epithelioid cell tumor"+" Pulmonary "+"adenocarcinoma" was used to search CNKI and Wanfang Medical Database, but no relevant reports were found. Then, we changed the search term as "pulmonary malignant perivascular epithelioid cell tumor", and search for PubMed, Embase, Web of Science and Cochrane by combining the subject terms with "pulmonary malignant perivascular epithelioid cell tumor" and "PEComa" as subtopics. The language was Chinese or English and the search deadline was November 2020.Results:The patient, a 46-year-old male, was admitted to the hospital on August 20, 2020, due to "repeated cough and chest pain for more than 10 days, accompanied by rapid weight loss". Serology detected increased expression of lung non-small cell lung cancer related antigens. PET-CT showed a large mass of soft tissue density in the left thoracic cavity with an SUV value of 22.8. The postoperative pathological diagnosis was malignant PEComa mixed with adenocarcinoma and the lymph nodes were metastasized. Due to the detection of EGFR sensitive mutation, postoperative chemotherapy and targeted therapy were administered, and the current state was stable. A total of 12 cases of pulmonary malignant PEComa were retrieved in the literature, which were common in middle-aged and elderly people. They usually presented with cough or chest tightness. Chest CT mostly showed round masses with clear boundaries, and 8 cases had metastasis to mediastinal lymph nodes and other organs.Conclusions:Pulmonary malignant PEComa is rare. It is the first report of the same mass with lung primary adenocarcinoma. The tumor progresses rapidly. Complete surgical resection of the lesion and lymph node dissection are more appropriate treatment strategies, supplemented by postoperative chemotherapy and targeted therapy. For cases diagnosed as pulmonary PEComa, long term follow-up should be performed, even if the pathological diagnosis is benign.