肺原始神经外胚层瘤2例并文献复习  

Pulmonary Primitive Neuroectodermal Tumor:2 Cases of Reports and Review of the Literatures

作　　者：汪庚明[1] 张雷[2] 周燕[1] 孙谦[1] 汪朝歌 陈丽丽 朱博[3] WANG Gengming;ZHANG Lei;ZHOU Yan;SUN Qian;WANG Chaoge;CHEN Lili;ZHU Bo(Department of Radiation Ontology,Tile First Affiliated Hospital of Bengbu Medical College,Bengbu 233000,China;Department of Thoracic Surgery;Department of Pathology)

机构地区：[1]蚌埠医学院第一附属医院肿瘤放疗科,安徽蚌埠233000 [2]蚌埠医学院第一附属医院肿瘤胸外科,安徽蚌埠233000 [3]蚌埠医学院第一附属医院肿瘤病理科,安徽蚌埠233000

出　　处：《沈阳医学院学报》2018年第6期517-519,共3页Journal of Shenyang Medical College

摘　　要：目的:探讨肺原始神经外胚层瘤(primitive neuroectodermal tumor,PNET)的临床病理学特征、诊断、治疗及预后。方法:对2例肺PNET患者组织病理学检查、免疫组化染色结果等进行总结,并复习相关文献。结果:例1,男,66岁,肿物位于右下肺;例2,女,12岁,肿物位于左肺尖。HE染色示小圆细胞恶性肿瘤,未见Homer-Wright菊形团结构或假菊形团状排列。2例免疫组化结果示CD99、Vim均阳性,CK、EMA、Syn、CD34均阴性。例1就诊我科后,病情迅速恶化,出现呼吸困难、意识丧失,遂自动出院,于出院后1周内死亡。例2先后行全身化疗8周期,肿瘤曾一度有所缩小,目前疾病进展,出现呼吸困难,行对症支持治疗;目前存活,但一般情况较差。通过对相关文献检索显示,肺PNET临床表现无特异性,以胸痛、胸闷为主,影像学多为较大的软组织块影,伴或不伴有胸腔积液;治疗方式包括手术、放化疗等;虽经治疗,但总体预后不佳。结论:肺PNET是一种较为罕见的高度恶性神经系统肿瘤,确诊主要依靠病理学检测。预后不良,综合治疗可提高疗效。Objective： To investigate the clinicopathological features, diagnosis, treatment and prognosis of pulmonary primitive neuroectoderaml tumor （PNET）. Method： Results of HE and immunohistoehemieal staining of 2 patients with pulmonary PNET were summarized and relevant literatures were reviewed. Results： Case 1 was a 66 years old man who had a trmor under the right lower lung.Case 2 was a 12 years old girl who had a tumor in the apex of left lung. HE staining showed the existence of malignant small round cell tumor without Homer-Wright rosettes or pseudorosettes, hnmunohistoehemieal staining showed that CD99 and Vim were positive, and CK, EMA, Syn and CD34 were negative. Case 1 had rapid deterioration with the occurrence of dyspnea and loss of consciousness, and he died within 1 week post-discharge. Case 2 received systemic chemotherapy for 8 cycles, and the size of the tumor reduced. At present, dyspnea occurred and symptomatic treatment was given. Now, she is alive except the poor general condition. Related literatures show that the symptoms of puhnonary PNET are non-specific, with chest pain and chest tightness as the main features. Chest CT revealed a large soft tissue mass with or without pleural effusion. The treatment methods include surgery, radiotherapy and chemotherapy. The overall prognosis is poor. Conclusions： Puhnonary PNET is a relatively rare malignant tumor of the nervous system, and the diagnosis mainly depends on pathological examination. In addition, it has poor prognosis and comprehensive treatment can improve the curative effect.

关 键 词：原始神经外胚层瘤 病理学 免疫组织化学 

分 类 号：R734.2[医药卫生—肿瘤]