罕见胸腔软组织血管肉瘤一例并文献复习

Rare angiosarcoma of thoracic soft tissue:a case report and literature review

目的结合文献复习胸腔软组织血管肉瘤的临床表现、病理形态、影像表现、治疗方法及预后。方法回顾性分析陕西省结核病防治院2020年5月8日收治的1例胸腔软组织血管肉瘤患者的临床资料、诊疗经过及随访情况,并进行文献复习。以"血管肉瘤"为检索词通过中国知网、万方数据库对中文文献进行检索,以"lung angiosarcoma""hemangiosarcoma"为检索词对PubMed数据库进行检索,检索到国内相关文献56篇,国外相关文献21篇,选取资料完整的12篇病例报告中的15例患者进行总结,结合本研究收集的患者,对其临床特征、诊断及治疗情况进行分析。结果此例患者为男性,34岁,以"咯血1个月"为主要症状,分别于2020年5月9日和14日经介入栓塞治疗2次,止血效果不佳,于2020年6月12日开胸行左侧胸腔病灶清除及左肺下叶切除术,术后胸腔持续出血,2020年6月24日行开胸探查止血及胸膜活检术,术后胸腔仍大量出血,2021年2月1日行开胸探查及胸廓成形术,术后免疫组化血小板-内皮细胞黏附分子、人造血祖细胞抗原、波形蛋白、细胞增殖标志指数-67、ETS相关基因阳性,确诊血管肉瘤,并转至肿瘤专科医院输血治疗15d后死亡。检索文献后获得15例患者,加上此例,共16例患者。其中,男性10例,女性6例,中位年龄54岁;临床症状:咯血8例,胸闷气短6例,咳嗽7例,胸痛4例;并发肺结核及结核性脓胸1例,并发肺癌1例,并发胸腔积液5例;单侧肺部病变7例(左肺3例,右肺4例),双肺病变9例;血小板-内皮细胞黏附分子阳性者16例,人造血祖细胞抗原阳性者9例,波形蛋白阳性者4例,细胞增殖标志指数-67阳性者3例,血浆凝血因子Ⅷ阳性者7例,ETS相关基因阳性者2例,弗里德白血病病毒插入位点1阳性者2例;16例患者全部由病理组织检查确诊,其中穿刺活检3例,胸腔镜活检13例;10例接受手术治疗,3例接受化疗,2例接受手术+化疗,1例放弃治疗;13例患者死亡,3例失访,平均生存时间(7.2±2.1)个月。结论胸腔软组织血管肉瘤是非常罕见的软组织来源的恶性肿瘤,预后差,治疗方法包括手术、放化疗及分子靶向治疗。

Objective To review the clinical manifestations,pathological morphology,imaging findings,treatment and prognosis of thoracic soft tissue angiosarcoma.Methods The clinical data,diagnosis,treatment and follow-up of a patients with pleural soft tissue angiosarcoma admitted to Shaanxi Tuberculosis Control Hospital on May 8,2020 were retrospectively analyzed with review of literatures.Taking"angiosarcoma"as the search term,the Chinese literature was searched through the CNKI and Wanfang,and"lung angiosarcoma"and"hemangiosarcoma"as the search terms,56 domestic and 21 foreign related literatures were retrieved through the PubMed.Fifteen patients with complete data in 12 case reports were selected and summarized.The clinical characteristics,diagnosis and treatment of them were analyzed with the reported case.Results The patient was34 years old,male,and the main symptom was"hemoptysis for 1 month".The hemostatic effect was poor after interventional embolization on May 9 and 14,2020.On June 12,2020,the left thoracic focus was removed and the left lower lobe of lung was resected,the thoracic cavity continued to bleed after operation.On June 24,2020,the thoracic exploration,hemostasis and pleural biopsy were performed.There was still a lot of bleeding in the thoracic cavity after operation.On February 1,2021,thoracotomy exploration and thoracoplasty were performed.After operation,immunohistochemistry platelet-endothelial cell adhesion molecule,artificial hematopoietic progenitor cell antigen,vimentin,cell proliferation marker index-67 and ETS-related genes were positive,and the patient was diagnosed as Angiosarcoma and transferred to tumor hospital for blood transfusion.Half a month later,the patient died.Of the 16 patients(including 15 obtained from the literature),10 were male and 6 were female,and the average age was 54 years.Clinical symptoms:hemoptysis in 8 cases,chest tightness and shortness of breath in6 cases,cough in 7 cases and chest pain in 4 cases;1 case was complicated with pulmonary tuberculosis and tuberculous empyema,1 case was complicated with lung cancer.Of the 7 unilateral pulmonary cases:3 in left lung and 4 in right lung.Bilateral lung lesions were found in 9 cases,platelet-endothelial cell adhesion molecule was positive in 16 cases,artificial hematopoietic progenitor cell antigen was positive in 9 cases,vimentin was positive in4 cases,cell proliferation marker index-67 was positive in 3 cases,plasma coagulation factorⅧwas positive in7 cases,ETS related gene was positive in 2 cases and Fried leukemia virus insertion site 1 was positive in 2 cases.All the 16 patients were confirmed by pathological examination,3 were puncture biopsy and 13 were thoracoscopic biopsy;10 cases received surgical treatment,3 cases received chemotherapy,2 cases received surgery+chemotherapy,and 1 case gave up treatment;13 cases died and 3 cases were lost in follow-up.The average survival time was(7.2±2.1)months.Conclusion Thoracic soft tissue angiosarcoma is a very rare malignant tumor from soft tissue with poor prognosis.The treatment methods include surgery,radiotherapy and chemotherapy,and molecular targeted therapy.