颈部实体型血管瘤样纤维组织细胞瘤2例并文献复习  

Solid angiomatoid fibrous histiocytoma of the neck:A report of 2 cases and literature review

作　　者：王凡[1,2] 袁静萍 何惠华[1] 张世英[1] 柯晓康[1] 徐励 叶成林 陈芳芳 WANG Fan;YUAN Jingping;HE Huihua;ZHANG Shiying;KE Xiaokang;XU Li;YE Chenglin;CHEN Fangfang(Department of Pathology,Renmin Hospital of Wuhan University,Wuhan 430060;Department of Pathology,Luotian Wanmizhai Hospital,Huanggang Hubei 438600,China)

机构地区：[1]武汉大学人民医院病理科,武汉430060 [2]罗田县万密斋医院病理科,湖北黄冈438600

出　　处：《临床与病理杂志》2023年第9期1758-1764,共7页Journal of Clinical and Pathological Research

基　　金：武汉市科技计划项目(2017060201010172);武汉大学人民医院引导基金(RMYD2018M27)。

摘　　要：血管瘤样纤维组织细胞瘤(angiomatoid fibrous histiocytoma,AFH)是一种少见的分化未定的软组织肿瘤。本研究回顾性分析武汉大学人民医院分别于2021年9月和12月收治的2例颈部实体型AFH患者的临床资料,并对其组织形态、免疫组织化学染色及分子遗传学检测结果进行分析。2例均为老年男性,病例1因CT发现颈部实性包块2 d入院,病例2因发现颈部皮下豌豆大小包块2年,近来包块逐渐增大入院。2例均行单纯手术切除术,大体检查见肿瘤切面灰白,质韧,均未见明显出血性囊腔。光镜下肿瘤周边由纤维性假包膜构成,包膜下可见淋巴浆细胞套,肿瘤组织主要由梭形及卵圆形细胞构成,呈结节状、片状聚集,肿瘤细胞分布疏密不均,形态温和,偶见核分裂象(1~2/10 HPF),未见出血性囊腔或假血管瘤样间隙。免疫表型:间变性淋巴瘤激酶(anaplastic lymphoma kinase,ALK)(+),上皮膜抗原(epithelial membrane antigen,EMA)(+),CD99(+),结蛋白(Desmin)(−),SOX10(−),Ki-67(+,热点区约8%)。荧光原位杂交(fluorescence in situ hybridization,FISH)检测示病例1 EWSR1断裂。病例1随访11个月,无复发及转移;病例2随访19个月,无复发及转移。实体型AFH罕见,发生于颈部的实体型AFH未见文献报道,本研究为首次报道。光镜下实体型AFH因不含特征性的假血管瘤样间隙,容易误诊。肿瘤周围的淋巴浆细胞套对实体型AFH的诊断具有提示意义。因实体型AFH尚无特异的免疫表型,FISH检测到EWSR1断裂对诊断帮助较大。2例患者均未做任何辅助治疗,随访期间均无瘤生存。Angiomatoid fibrous histiocytoma(AFH)is a rare undifferentiated soft tissue tumor.This study analyzed the clinical data of 2 cases of cervical solid AFH admitted to Wuhan University People’s Hospital in September and December 2021.Histomorphology,immunohistochemical staining,and molecular genetic test were analyzed.Both cases were elderly males.Case 1 was admitted due to a solid mass in the neck on CT for 2 days,while case 2 was admitted due to the discovery of a subcutaneous pea-sized mass in the neck 2 years ago,which had gradually increased in size recently.Both cases underwent simple surgical resection,and the tumor was found to have a gray-white and tough cut surface.No obvious hemorrhagic cystic cavity was observed in either case.Microscopically,the periphery of the tumor was composed of a fibrous pseudocapsule with a lymphoid plasma cell sleeve.The tumor tissues mainly consisted of spindle and oval cells clustered in nodular or sheet-like shapes.The distribution of tumor cells was uneven,with mild morphology and occasional mitotic figures(1−2/10 HPF),and no hemorrhagic cystic cavities or pseudoangiomatous spaces were observed.Immunophenotypes:anaplastic lymphoma kinase(ALK)(+),epithelial membrane antigen(EMA)(+),CD99(+),desmin(−),SOX10(−),Ki-67(+,hot spot approximately 8%).Fluorescence in situ hybridization(FISH)detection showed EWSR1 cleavage in case 1.Case 1 was followed up for 11 months without recurrence or metastasis;case 2 was followed up for 19 months without recurrence or metastasis.Solid-type AFH is rare and,solid-type AFH occurring in the neck has not been reported in the literature.This study is the first to report on solid-type AFH occurring in the neck.Microscopically,solid-type AFH is prone to misdiagnosis due to its lack of characteristic pseudoangiomatous spaces.The lymphatic plasma cell sheath around the tumor is suggestive for the diagnosis of solid-type AFH.Due to the lack of a specific immune phenotype for solid-type AFH,FISH detection of EWSR1 breakage is of great diagnostic va

关 键 词：颈部 实体型 血管瘤样纤维组织细胞瘤 临床病理特征 分子检测 

分 类 号：R739.91[医药卫生—肿瘤]