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成人肺母细胞瘤的诊断与多模态治疗:18例分析并文献复习

Diagnosis and multi-modality treatment of adult pulmonary plastoma: Analysis of 18 cases and review of literature.

作者信息

Wang Yun-Xi, Zhang Jing, Chu Xiang-Yang, Liu Yi, Li Fang, Wang Zhan-Bo, Wei Li-Xin

机构信息

Department of Thoracic Surgery, General Hospital of PLA, Beijing 100853, China.

Department of Thoracic Surgery, General Hospital of PLA, Beijing 100853, China.

出版信息

Asian Pac J Trop Med. 2014 Feb;7(2):164-8. doi: 10.1016/S1995-7645(14)60015-8.

DOI:10.1016/S1995-7645(14)60015-8
PMID:24461533
Abstract

The clinical data of 18 patients with PB from April 1989 to April 2013 was analyzed retrospectively, including 11 men and 7 women, aged 45 and 76 years old (mean 53 years). There were 12 cases of PB occurring in right lung and other cases in left lung. Among them, 3 patients had no symptoms, and 15 patients displayed symptoms of cough, chest pain, asthenia or minor haemoptysis. Overall, 11 patients had a preoperative diagnosis of lung cancer, 7 patients were preoperatively diagnosed as the other diseases, which included lung benign tumor (n=5) and mediastinal mass (n=2). All patients received a radical resection. Six patients received postoperative cisplatin-based chemotherapy, and two patients received postoperative irradiation with the dose of 55 Gy. Histologically, 14 cases of 18 patients had biphasic pulmonary blastoma and four cases had well differentiated fetal adenocarcinoma. A total of 12 patients died in a period of 6-36 months after operation, and 1 case was lost after 2 years of follow up. The median survival time was 19 months. PB is a rare primary lung malignant embryonal neoplasm. Despite its assumed embyonal origin, the tumor has a predilection for adults. A preoperative correct diagnosis is very difficult in spite of modern diagnostic imaging and biopsy techniques. Surgical resection is the main method for diagnosis and treatment. Postoperative chemotherapy or irradiation can help eliminate tumor remnants. Its prognosis is very poor, especially for the biphasic type.

摘要

回顾性分析了1989年4月至2013年4月期间18例肺母细胞瘤患者的临床资料,其中男性11例,女性7例,年龄45至76岁(平均53岁)。12例肺母细胞瘤发生于右肺,其余发生于左肺。其中,3例患者无症状,15例患者表现为咳嗽、胸痛、乏力或少量咯血。总体而言,11例患者术前诊断为肺癌,7例患者术前诊断为其他疾病,包括肺良性肿瘤(5例)和纵隔肿块(2例)。所有患者均接受了根治性切除。6例患者术后接受了以顺铂为基础的化疗,2例患者术后接受了55 Gy的放疗。组织学上,18例患者中有14例为双相性肺母细胞瘤,4例为高分化胎儿腺癌。共有12例患者在术后6至36个月内死亡,1例患者在随访2年后失访。中位生存时间为19个月。肺母细胞瘤是一种罕见的原发性肺恶性胚胎性肿瘤。尽管其起源于胚胎,但该肿瘤好发于成年人。尽管有现代诊断成像和活检技术,术前正确诊断仍非常困难。手术切除是主要的诊断和治疗方法。术后化疗或放疗有助于清除肿瘤残余。其预后非常差,尤其是双相型。

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