摘要
目的 探讨血管肉瘤(AS)肺转移的临床病理及免疫表型特征,结合文献探讨其鉴别诊断及治疗方案。方法 收集2018年1月至2023年12月在西安交通大学第一附属医院病理科确诊的AS肺转移3例病例的临床及影像资料,EnVision两步法行免疫组织化学染色,荧光原位杂交检测SS18基因易位情况,并复习文献探讨新兴治疗方法。结果 3例均为男性,年龄45~73岁。临床表现为阵发性胸闷、气短、间断咯血、咳嗽、咳痰、右髋部疼痛不适等。影像学上肺部均出现多发大小不等、边界较清的高密度结节或条索灶。行活检病理组织形态表现为:肺组织内见肿瘤成片状浸润性生长,其间可见大小不等腔隙;细胞呈短梭形或上皮样,胞质嗜酸性或双色性,界限不清;细胞核呈泡状,部分可见核仁;肿瘤坏死明显,易见核分裂像及病理性核分裂像。免疫表型:3例样本肿瘤细胞Vim、CD34、CD31、ERG、FLI-1、INI1、CD99阳性;P40、SMA、Desmin、ALK、CD68、E-cadherin、S100、HMB45、MelanA、WT-1、D2-40、BRG-1、HHV8、LCA、CK、CK7、TTF1、CK5/6、EMA阴性;Ki67阳性率55%~65%。2例荧光原位杂交未检测到SS18基因易位。确诊后2例行姑息治疗,1例行化疗联合靶向治疗。随访1例带瘤生存3个月,2例分别于9、4个月后因病死亡。结论 病理组织形态及一组包括内皮细胞标记的免疫组化检测,有助于明确AS肺转移诊断。靶向和免疫治疗是有前景的治疗方法,但需进一步验证。
Objective To investigate the clinical,pathological,and immunophenotypic character⁃istics of angiosarcoma(AS)with pulmonary metastases,and explore its differential diagnosis and treatment strategies in combination with literature.Methods Clinical and imaging data from 3 cases of AS with pulmo⁃nary metastases were collected and confirmed by the Department of Pathology at the First Affiliated Hospital of Xi'an Jiaotong University from January 2018 to December 2023.EnVision two⁃step immunohistochemical stain⁃ing,and fluorescence in situ hybridization(FISH)studies were conducted to detect SS18 gene translocations.Additionally,relevant literature was reviewed.Results All three cases were males,aged 45 to 73 years.The clinical symptoms included paroxysmal chest tightness,shortness of breath,intermittent hemoptysis,cough,sputum,and pain and discomfort in the right hip.Imaging scans displayed multiple high⁃density nodules or lin⁃ear lesions of varying sizes with clear boundaries in the lungs.The pathological morphology of the biopsy tissue showed patchy infiltrative growth of tumors in the lung tissue,with varying sizes of cavities visible between them.The cells were short spindle shaped or epithelioid,with eosinophilic or bichromatic cytoplasm and unclear boundaries.The nucleus was vesicular,with some visible nucleoli.The tumor necrosis was obvious,mitotic im⁃ages and pathological mitotic images were easily visible.Immunophenotype:Three samples showed that tumor cells were positive for Vim,CD34,CD31,ERG,FLI⁃1,INI1,and CD99;negative for P40,SMA,Desmin,ALK,CD68,E⁃cadherin,S100,HMB45,MelaNA,WT⁃1,D2⁃40,BRG⁃1,HHV8,LCA,CK,CK7,TTF1,CK5/6,EMA.The positive rate of Ki67 was 55%~65%.SS18 gene translocation was not detected in two cases by FISH.After diagnosis,2 cases were treated with palliative care,one case was treated with chemo⁃therapy and targeted therapy.One case with the tumor survived for 3 months,while two cases died of illness af⁃ter 9 and 4 months,respectively.Conclusion Pathological morphology and a panel of immunohistochemical markers,including endothelial cell markers,are helpful for diagnosing AS with pulmonary metastases.Targeted and immunotherapy are promising treatment strategies,but further validation is needed.
作者
袁兴娥
贾琪琪
高姗
杨喆
张冠军
刘希
YUAN Xinge;JIA Qiqi;GAO Shan;YANG Zhe;ZHANG Guanjun;LIU Xi(Department of Pathology,the First Affiliated Hospital of Xi'an Jiaotong University,Xi'an,Shaanxi,China,710061;Department of Pathology,Ankang People's Hospital,Ankang,Shaanxi,China,725000)
出处
《分子诊断与治疗杂志》
2025年第11期2053-2055,2059,共4页
Journal of Molecular Diagnostics and Therapy
基金
国家自然科学基金项目(81902414)
陕西省重点产业创新链(群)-社会发展领域项目(2020ZDLSF03-04)。
关键词
血管肉瘤肺转移
病理诊断
免疫组织化学检测
Angiosarcoma with pulmonary metastases
Pathologic diagnosis
Immunohistochemical testing
