15例原发性肺肉瘤样癌临床病理观察

Clinicopathology observation of primary pulmonary sarcomatold carcinoma:15 cases

目的探讨原发性肺肉瘤样癌(PPSC)的临床病理特征、诊断及鉴别诊断,提高对本病的认识。方法回顾性分析15例经病理确诊为PPSC患者的临床资料、病理特点及分子、免疫表型。结果15例中男13例,女2例,年龄56~76岁,中位年龄66岁。病变位于左肺上叶8例,左肺下叶6例,右肺中叶1例。临床主要表现为咳嗽、咳痰、痰中带血、胸痛。男性患者中10例有30年以上吸烟史。所有患者术前均行胸部CT平扫+增强检查,提示肺内实质性肿块,肿块周边厚薄不均环形强化,肿块中央强化不明显或不均强化。病理类型:梭形细胞癌6例,多形性癌4例,巨细胞癌2例,癌肉瘤2例,肺母细胞瘤1例,肿瘤均由上皮成分和肉瘤样成分组成。免疫组化结果显示15例上皮角蛋白CK(+),7例上皮细胞膜抗原EMA(+),10例波形蛋白VIM(+)。其中10例行肺癌常见相关基因检测,4例出现MET14跳跃突变,3例EGFR L858R基因突变,2例KARS G13D突变,1例BRAF V600E突变。15例均行肺叶切除手术,13例术后行辅助化疗,6例辅以局部放疗。术后随访8~50个月,3例失访,术后3年生存4例。结论PPSC是一类少见的分化差的非小细胞性肺癌,临床表现和影像学检查缺乏特异性,确诊依赖术后病理组织学检查及免疫组化,需与多种疾病鉴别。此病较一般肺癌更具有侵袭性,预后差。

Objective To investigate the clinical pathological feature of primary pulmonary sarcomatold carcinoma and to make a further understandine of the disease.Methods Data including clinical manifesation,pathological findings,molecular detection and immunophenotyping with pathologically confirmed primary pulmonary sarcomatold carcinoma was retrospectively analyzed.Results 15 patients with PPSC were identified(13 men and 2 women,age ranged 56-76 years,median age 66 years).The tumor were located in the left lobus superior(8 cases),lobus inferior(6 cases),and the right lobus medius(1 case).The main clinical symptoms was cough,sputum,bloody sputum,chest pain.Among the 13 males,10 had smoking history of more than 30 years,and 2 females had no smoking history.All cases presented with a spheroid solid lung mass.All tumor showed mild enhancement similar to that of the surrounding musculature after contrast enhancement,and inhomogeneous central low-attenuation areas were seen in 15 patients.Pathological pattern:6 cases spindle cell carcinoma,4 cases pleomorphic carcinoma,2 cases giant cells carcinoma,2 cases carcinosarcoma,1 case pulmonary blastoma.The tumors were composed of both carcinomatous and sarcomatous elements.Immunohistochemistry showed that CK was all positive,EMA was positive in 7,VIM was positive in 10 of 15 cases.10 patients were tested for common related genes of lung cancer,4 patients had MET14 jump mutation,EGFR L858R gene mutation occurred in 3 cases,KARS G13D gene mutation in 2 cases,and BRAF V600E mutation in 1 case.All 15 patients underwent lobectomy,13 underwent adjuvant chemotherapy,and 6 underwent local radiotherapy.Postoperative follow-up was 8 to 50 months,3 cases were lost,and 4 cases were survival 3 years after the surgery.Conclusion Pulmonary sarcomatold carcinoma is a rare histologic subtype of non-small cell lung cancer.Compared with other NSCLC,there is no special clinical and imageing characteristics.Its definite diagnosis relies on postoperative pathological analysis and immunohistochemical staining,and PSC needs to be diatinguished from a variely of disease.PPSC is more aggressive and poor prognosis.