散发型多原发胃肠间质瘤临床病理特征及预后

Clinicopathological characteristics and prognosis of patients with sporadic multiple primary gastrointestinal stromal tumors

目的探讨散发型多原发胃肠间质瘤(GIST)的临床病理特征及预后。方法采用回顾性队列研究方法,病例纳入标准:(1)术后病理确诊为GIST;(2)单发或散发型多原发者(散发型GIST定义为除家族性和综合征相关GIST外的原发GIST,多原发GIST定义为同一患者GIST个数≥2个,且无证据表明肿瘤为复发或转移);(3)临床病理资料完整者。收集2010年1月至2020年12月期间,于华中科技大学同济医学院附属协和医院行外科手术治疗的原发GIST患者完整病例资料,排除肿瘤复发或伴远处转移、以及伴发其他恶性肿瘤者后,根据GIST原发数量将患者分为散发性多原发GIST组和单原发GIST组。观察指标包括两组临床和肿瘤病理数据及预后的比较。结果本研究共纳入1200例原发GIST患者,男性628例(52.3%),女性572例(47.7%),发病中位年龄58(19~93)岁。1165例(97.1%)为单发GIST,35例(2.9%)为散发型多原发GIST。35例散发型多原发GIST组中,有3例(8.6%)首发症状为反酸,比例高于单发GIST组(22/1165,1.9%),差异有统计学意义(χ^(2)=7.437,P=0.006)。两组患者其他临床特征的差异无统计学意义(均P>0.05)。散发型多原发GIST组患者共包含80个原发肿瘤。与单发GIST组相比,散发型多原发GIST组肿瘤原发于胃的比例更高[87.5%(70/80)比59.1%(689/1165)]、组织学为梭形细胞型的比例更低[85.0%(68/80)比93.7%(1092/1165)]、CD34阳性比例更高[97.5%(78/80)比87.6%(1021/1165)]、肿瘤最大径更小[最大径≤2.0 cm:61.2%(49/80)比28.8%(335/1165)]、核分裂象更小[≤5个/50高倍视野(HPF):93.8%(75/80)比74.5%(868/1165)]、危险度更低[极低危:60.0%(48/80)比23.3%(271/1165)],差异均有统计学意义(均P<0.05)。散发型多原发组和单发GIST组的3年无复发生存率(RFS)分别为96.6%和89.3%(P=0.160),3年总体生存率(OS)分别为100.0%和92.8%(P=0.088),差异均无统计学意义(均P>0.05)。结论散发型多原发GIST以多个肿瘤同时原发于胃最为常见。与单发GIST相比,散发型多原发GIST肿瘤最大径和核分裂象较小,两组患者预后相当。

Objective To investigate the clinicopathological characteristics and prognosis of sporadic multiple primary gastrointestinal stromal tumor(GIST).Methods A retrospective cohort study was conducted.Case inclusion criteria:(1)postoperative pathological diagnosis of GIST;(2)primary GIST with single lesion or sporadic multiple primary GIST(sporadic GIST was defined as primary GIST other than familial and syndrome⁃related GIST,and multiple primary GIST was defined as the number of primary GISTs in the same patient≥2);(3)patients with complete clinicopathological data.Those with tumor recurrence or distant metastasis,and with other malignancies were excluded.Medical records of patients with primary GIST who underwent surgical resection in the Union Hospital,Tongji Medical College,Huazhong University of Science and Technology from January 2010 to December 2020 were collected.Patients were divided into sporadic multiple primary GIST group and single primary GIST group according to the number of primary GIST lesions.The clinicopathological data and prognosis of the two groups were observed and compared.Results A total of 1200 patients with primary GIST were enrolled in this study,including 628 males(52.3%)and 572 females(47.7%),with a median onset age of 58(19⁃93)years.Among them,1165 cases(97.1%)were sporadic primary GIST with single lesion;35 cases(2.9%)were sporadic multiple primary GIST.Among 35 cases of sporadic multiple primary GIST,3 cases(8.6%)had acid reflux as the first symptom,which was higher than the single primary GIST group(22/1165,1.9%)(χ^(2)=7.437,P=0.006).There were no significant differences in other clinical characteristics between the two groups(all P>0.05).Patients in the sporadic multiple primary GIST group contained a total of 80 primary tumors.Compared with the single primary GIST group,the sporadic multiple primary GIST group had a higher proportion of tumors originating in the stomach[87.5%(70/80)vs.59.1%(689/1165)],lower proportion of spindle cell in histology[85.0%(68/80)vs.93.7%(1092/1165)],higher proportion of positive CD34[97.5%(78/80)vs.87.6%(1021/1165)],smaller maximum diameter[maximum diameter≤2.0 cm:61.2%(49/80)vs.28.8%(335/1165)],lower mitotic rate[≤5/50 high⁃power fields(HPF):93.8%(75/80)vs.74.5%(868/1165)],lower risk of recurrence[60.0%(48/80)vs.23.3%(271/1165)],and the differences were all statistically significant(all P<0.05).The 3⁃year recurrence⁃free survival rate in the sporadic multiple primary group and the single primary GIST group was 96.6%and 89.3%respectively(P=0.160),and the 3⁃year overall survival rate was 100.0%and 92.8%,respectively(P=0.088).Conclusions The most common type of sporadic multiple primary GIST is multiple tumors originating in the stomach at the same time.Compared with primary GIST with single lesion,sporadic multiple primary GIST presents smaller maximum diameter and lower mitotic rate.The prognosis of patients between two groups is not significantly different.