多部位原发性粒细胞肉瘤1例  

Multisite Primary Granulocytic Sarcoma:a Case Report

作　　者：田云生[1] 林莉 廖娟彬 陈华[1] 刘臻[1] TIAN Yun-sheng;LIN Li;LIAO Juan-bin;CHEN Hua;LIU Zhen(PETCT Center,Department of Nuclear Medicine,Ganzhou People’s Hospital(Ganzhou Hospital of Southern Hospital),Ganzhou341000,China)

机构地区：[1]赣州市人民医院(南方医院赣州医院)核医学科PETCT中心,江西赣州341000

出　　处：《实用临床医学（江西）》2023年第5期43-47,共5页Practical Clinical Medicine

摘　　要：目的探讨多部位原发性粒细胞肉瘤(GS)的临床、影像特点和诊治方法。方法对1例多部位原发性GS的临床表现、影像诊断及治疗进行回顾性分析。结果患者,女,72岁,因颈部及腋下无痛性包块渐进性增大,颈部包块大小约为5.0 cm,质中,表面无发热;影像和PETCT示回肠、腹腔淋巴结、左乳腺、子宫、右侧腹壁多发肿块和结节灶。送检病理组织进行AML相关基因测序,检测到Ⅱ类突变FLT3 exon20 p.D839G突变(47.8%)及Ⅲ类突变ARID1B exon1(36.1%),且22号染色体拷贝数增加。免疫组织化学示:CD117(+),CD20(-),CD3(-),CD34(部分细胞+),CK(-),Ki-67(约80%+),BCL2(+),CD99(-),CK20(-),CK7(-),STAT6(-),CD31(+),D0G-1(-),D2-40(-),Fli-1(+),MPO(+),CR(-),MC(-),WT1(-),支持GS诊断。使用维奈克拉100 mg d1,200 mg d2,400 mg d3-14,阿扎胞苷110 mg d1-7治疗。化疗结束4个月,CT复查示回肠肿块明显缩小,左乳腺肿块和腹腔淋巴结缩小,子宫结节不明显。结论GS缺乏特异性的临床表现,多部位更为罕见,术前诊断有一定困难,确诊主要依靠病理学和免疫组织化学手段。PETCT对病灶的显示敏感、准确,对临床诊断和评估有一定的帮助。GS预后差,需尽早诊断并按标准AML化疗方案进行治疗。Objective To investigate the clinical manifestations,imaging features,diagnosis and treatment of multisite primary granulocytic sarcoma(GS).Methods The clinical manifestations,imaging diagnosis and treatment of 1 case of multisite primary GS were retrospectively analyzed.Results The 72-year-old female patient presented with painless mass in the neck and armpit that had gradually enlarged.The neck mass was about 5.0 cm in size without surface fever.PETCT scan showed multiple masses and nodules in the ileum,abdominal lymph nodes,left breast,uterus,and right abdominal wall.Sequencing of AML-related genes in the pathological tissues detected classⅡmutation FLT3 exon20 p.D839G mutation(47.8%)and classⅢmutation ARID1B exon1(36.1%),with increased copy number of chromosome 22.Immunohistochemistry showed that the cells were CD117(+),CD20(-),CD3(-),CD34(some cells+),CK(-),Ki-67(about 80%+),BCL2(+),CD99(-),CK20(-),CK7(-),STAT6(-),CD31(+),D0G-1(-),D2-40(-),Fli-1(+),MPO(+),CR(-),MC(-)and WT1(-),which supported the diagnosis of GS.The patient was treated with venclexta venecola(100 mg d1,200 mg d2 and 400 mg d3-14)and azacitidine(110 mg d1-7).At 4 months after the end of chemotherapy,CT reexamination showed that the ileum mass,left breast mass and abdominal lymph nodes were reduced without obvious uterine nodules.Conclusion GS lacks specific clinical manifestations and is rare in multiple sites.It is difficult to diagnose preoperatively,and its definite diagnosismainly determined by pathology and immunohistochemistry.PETCT is sensitive and accurate to display lesions,and is therefore helpful to clinical diagnosis and evaluation.GS is associated with a poor prognosis and requires early diagnosis and treatment according to standard AML chemotherapy regimens.

关 键 词：粒细胞肉瘤 髓系肉瘤 PETCT 

分 类 号：R733[医药卫生—肿瘤]