Li M N, Bao M L, Gong Q X, Zhu Y, Li X, Song G X, Li H X, Fan Q H
Department of Pathology, Jiangsu Province People's Hospital (the First Affiliated Hospital of Nanjing Medical University), Nanjing 210029, China.
Zhonghua Bing Li Xue Za Zhi. 2020 Aug 8;49(8):816-820. doi: 10.3760/cma.j.cn112151-20191219-00815.
To investigate the clinicopathological characteristics, genetic features, diagnosis and differential diagnosis of pulmonary artery intimal sarcoma (PAIS). Three cases of PAIS were collected from Jiangsu Province People's Hospital (from February 2016 to November 2019). The clinical data, imaging examination, morphology, immunostaining, and molecular changes were retrospectively analyzed. There were 1 male and 2 females (age: 32, 50, 60 years), who had symptoms of cough, asthma or chest tightness. Imaging findings indicated low density filling defects which were suspected as thrombus, embolism or myxoma. Grossly, the main tumor was located in the elastic arteries and their lobar branches, also extended into the atrium and ventricle, with lung parenchymal infiltration focally. Microscopically, tumor cells were predominantly composed of abundant spindle cells with obvious atypia and myxoid background, resembling fibroblastic or myofibroblastic differentiation. Active mitotic figures and necrosis could be seen in some areas. Immunohistochemical staining of vimentin was strongly positive, while pan-cytokeratin, S-100, desmin, Fli-1, CD31, SMA and ERG etc were variably positive only in focal areas. FISH detection showed amplification of MDM2 gene in three cases and EGFR gene in two cases. Metastatic lesions were found in one case by 18, 32 and 42 months after surgery respectively. There was no recurrence or metastasis in the other two cases. PAIS is one of exceptionally poor differentiated mesenchymal tumor that arises from the arterial intima of elastic pulmonary arteries. There was no definite differention in morphology. Gene detection shows amplification of MDM2 and EGFR gene. This tumor often has poor prognosis with aggressive behavior. Complete resection is the only effective therapeutic option. There is disagreement as to whether chemotherapy and radiotherapy can improve survival.
探讨肺动脉内膜肉瘤（PAIS）的临床病理特征、基因特征、诊断及鉴别诊断。收集江苏省人民医院2016年2月至2019年11月期间的3例PAIS病例。对其临床资料、影像学检查、形态学、免疫组化及分子改变进行回顾性分析。患者1例男性，2例女性（年龄分别为32岁、50岁、60岁），均有咳嗽、气喘或胸闷症状。影像学表现为低密度充盈缺损，怀疑为血栓、栓塞或黏液瘤。大体上，主要肿瘤位于弹性动脉及其叶分支，也可延伸至心房和心室，局部侵犯肺实质。显微镜下，肿瘤细胞主要由丰富的梭形细胞组成，具有明显异型性及黏液样背景，类似成纤维细胞或肌成纤维细胞分化。部分区域可见活跃的核分裂象及坏死。波形蛋白免疫组化染色呈强阳性，而广谱细胞角蛋白、S-100、结蛋白、Fli-1、CD31、平滑肌肌动蛋白和ERG等仅在局部区域呈不同程度阳性。荧光原位杂交检测显示3例MDM2基因扩增，2例EGFR基因扩增。1例患者术后分别于18个月、32个月和42个月发现转移灶。另外2例无复发或转移。PAIS是一种起源于弹性肺动脉内膜的低分化间叶性肿瘤。形态学上无明确的鉴别要点。基因检测显示MDM2和EGFR基因扩增。该肿瘤行为侵袭，预后通常较差。完整切除是唯一有效的治疗选择。对于化疗和放疗能否提高生存率尚无定论。
