Huang H J, Ye W B, Wen Y Q, Zhu Y, He J F
Department of Pathology, Affiliated Dongguan People's Hospital, Southern Medical University, Dongguan 523000, China.
Zhonghua Jie He He Hu Xi Za Zhi. 2021 May 12;44(5):468-473. doi: 10.3760/cma.j.cn112147-20201119-01104.
To analyze the pathogenesis, clinical manifestations, diagnosis, treatment and prognosis of pulmonary malignant perivascular epithelioid cell tumor (PEComa) with adenocarcinoma. 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. 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. 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.
分析肺恶性血管周上皮样细胞瘤（PEComa）合并腺癌的发病机制、临床表现、诊断、治疗及预后。2020年8月，南方医科大学附属东莞人民医院病理科诊断1例肺恶性PEComa合并腺癌。分析患者的临床资料、病理诊断、治疗方案及预后，并进行文献复习。首先，以“恶性血管周上皮样细胞瘤”+“肺”+“腺癌”检索中国知网和万方医学数据库，未发现相关报道。然后，将检索词改为“肺恶性血管周上皮样细胞瘤”，以“肺恶性血管周上皮样细胞瘤”和“PEComa”为副主题词，结合主题词检索PubMed、Embase、Web of Science和Cochrane数据库。语言限定为中文或英文，检索截止日期为2020年11月。患者为46岁男性，因“反复咳嗽、胸痛10余天，伴体重迅速下降”于2020年8月20日入院。血清学检测显示肺非小细胞肺癌相关抗原表达升高。PET-CT显示左胸腔巨大软组织密度肿块，SUV值为22.8。术后病理诊断为恶性PEComa合并腺癌，伴淋巴结转移。因检测到EGFR敏感突变，术后给予化疗及靶向治疗，目前病情稳定。文献中共检索到12例肺恶性PEComa，多见于中老年人。通常表现为咳嗽或胸闷。胸部CT大多显示边界清晰的圆形肿块，8例有纵隔淋巴结及其他器官转移。肺恶性PEComa罕见。这是同一肿块合并肺原发性腺癌的首次报道。肿瘤进展迅速。完整切除病变及清扫淋巴结是更合适的治疗策略，术后辅以化疗及靶向治疗。对于诊断为肺PEComa的病例，即使病理诊断为良性，也应进行长期随访。
