胰腺胃肠间质瘤4例报告及文献分析

Pancreatic gastrointestinal stromal tumors: A report of four cases and literature review

目的讨论胰腺胃肠间质瘤的临床病理特征、预后影响因素及基因突变特征。方法回顾性分析2010年5月至2018年10月西南医科大学附属医院确诊的4例胰腺胃肠间质瘤,并通过Pubmed及国内主要数据库检索国内外文献,进行文献复习及统计分析。结果国内外报道病例共55例,合并本次报道共59例。男性30例,女性29例,最常见临床首发症状为腹部疼痛或不适有38例(64.4%)、腹部肿块10例(16.9%)、体检发现或其他症状11例(18.7%);53例具有完整的影像学资料及组织学分型,影像学CT检查示肿瘤为不均匀低密度影,呈囊实性21例(39.7%)、呈实性20例(37.7%)、呈囊性12例(22.6%);最常见组织学类型是梭形细胞为主型44例(83.0%)、混合型7例(13.2%)、上皮细胞为主型2例(3.8%)。5年总存活率及无疾病存活率分别为79.7%及39.3%;单因素及多因素分析均显示,核分裂象>5个/50HPF是影响病人术后复发转移的独立因素(P均<0.05)。10例基因突变病人中9例(90.0%)为c-kit基因突变,1例(10.0%)为PDGFRα基因突变。结论胰腺胃肠间质瘤临床症状缺乏特异性,通常发现时肿瘤体积较大,多数病人具有高度风险性,且核分裂象>5个/50HPF是影响预后的独立因素,以c-kit基因突变多见。

Objective To investigate the clinicopathological features,prognosis and genes mutations of pancreatic gastrointestinal stromal tumors.Methods Four cases of pancreatic gastrointestinal stromal tumors were analyzed retrospectively.The cases were diagnosed in Affiliated Hospital of Southwest Medical University from May 2010 to October 2018.PubMed and main domestic databases were searched to retrieve the domestic and foreign literatures for literature review.Results Including our 4 cases,59 cases were reported,with 30 males and 29 females.The most common initial symptoms were abdominal pain or discomfort(38 cases,64.4%),abdominal mass(10 cases,16.9%)or health examination finds(11 cases,18.7%).A total of 53 cases were composed of complete imaging and histological type data.CT examination showed that the tumors were more uneven and low-density shadow,with solid-cystic(21 cases,39.7%),solid(20 cases,37.7%)or cystic(12 cases,22.6%).The most common histological types were spindle cell type(44 cases,83.0%),mixed type(7 cases,13.2%)or epithelioid cell type(2 cases,3.8%).The rates of 5-year overall survival(OS)and disease-free survival(DFS)were 79.7%and 39.3%respectively.By univariate and multivariate analysis,the main indicator for adverse outcome was mitoses>5/50 HPF(P<0.05).There were 9 cases(90.0%)with c-kit gene mutations and 1 case(10.0%)with PDGFRαgene mutation.Conclusion The clinical symptoms of pancreatic gastrointestinal stromal tumors are unspecific.The mitoses>5/50 HPF is an independent influencing factor of prognosis.Usually,the size of tumor is large and risk is high.C-kit gene mutations are common.