血管瘤样纤维组织细胞瘤10例临床病理及分子病理学研究  

Angiomatoid fibrous histiocytoma: a clinicopathological analysis of ten cases

作　　者：张荣君[1] 黄海建[2] 柳秋月 郑智勇[3] ZHANG Rong-jun;HUANG Hai-jian;LIU Qiu-yue;ZHENG Zhi-yong(Department of Pathology, Jinjiang City Hospital, Jinjiang 362200, China;Department of Pathology, Fujian Provincal Hospital, Provincial Clinical Medical College of Fujian Medical University, Fuzhou 350001, China;Department of Pathology, Fuzhou General Hospital of PLA, Fuzhou 350025, China)

机构地区：[1]晋江市医院病理科,福建晋江362200 [2]福建省立医院病理科,福州350001 [3]解放军福州总医院病理科,福州350001

出　　处：《诊断病理学杂志》2018年第1期28-31,36,共5页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨血管瘤样纤维组织细胞瘤(AFH)的临床病理学及分子病理学特征。方法对10例AFH进行病理形态学观察及免疫组化与分子病理学检测,随访治疗、预后并复习相关文献。结果 10例患者男女各5例,发病年龄6~76岁(平均33.7岁)。临床表现为皮下软组织肿块,咳嗽,或体检发现。肿瘤最大径1~7.5 cm(平均4.1 cm)。病理组织学表现,肿瘤有假包膜,包膜外具有"淋巴细胞套"。经典型(6/10)有实性区域和假血管腔隙;实性区瘤细胞梭形、圆形、卵圆形,呈束状、片状或不规则排列,轻度异型性,核分裂象少见(<2个/10 HPF)。假血管腔隙区域类似于血管瘤样,腔隙无血管内皮细胞衬覆。本组实体型4例,瘤细胞实性分布,无假血管腔隙或血管瘤样区域。免疫组化:肿瘤细胞vimentin(10/10)、a-SMA(3/10)、desmin(8/10)、CD68(5/10)、EMA(9/10)和CD99(8/10)(+),CD34、S-100、CD56、CD117、INI1、ALK1、HMB45和STAT6(-)。荧光原位杂交检测(FISH)10例均检测到EWSR1基因分离信号。结论 AFH是一种较为罕见的中间型软组织肿瘤,诊断依赖于形态学、免疫组化及分子病理学检测。治疗以手术完整切除为主,术后需要长期随访。Objective To investigate the clinicpathological and molecular features of angiomatoid fibrous histiocytoma（AFH）. Methods The clinical,histopathological,molecular findings,immunophenotype,treatment and prognosis in10 cases of AFH were evaluated with review of the relevant literatures. Results 5 cases were male,and 5 were female,aged from 6-76 years（mean 33. 7 years）. Clinical manifestations were subcutaneous soft tissue mass,cough,or discoverd by physical examination. The tumor size was 1 to 7. 5 cm（mean 4. 14 cm）. Microscopically, the histopathological findings were similar among soft tissue and other sites. The tumors had pseudocapsule with ＂lymphocyte sheath＂. Some cases were classic type（6/10）,which had solid region and pseudovascular lacunae areas. In solid region,the tumor cells were spindle,round,oval,with beam,sheet or irregular arrangement,and mild atypia. Mitotic fiqures were rare（about 〈 2/10 HPF）,with local visible multinucleated giant cells. At pseudovascular lacunae areas,it can be similar to hemangiomatous and without vascular endothelial cells lined. Some cases were the solid types（4/10）,characterized with the solid distribution,no hemangioma-like areas. Immunephenotyping,showed that tumor cells were positive for Vimentin（10/10）,a-SMA（3/10）,Desmin（8/10）,CD68（5/10）,EMA（9/10）,CD99（8/10）,and CD34,S100,CD56,CD117,INI1,ALK1,HMB45,and STAT6 were negative. EWSR1 gene isolation signal was detected in 10 cases by fluorescence in situ hybridization（10/10）. Conclusions AFH is a rare aggressive soft tissue tumor and its correct diagnosis relies on histopathologic features,immunohistochemical staining,and gene detection. The primary treatment is completely surgical resection and postoperative long-term follow-up is reserved.

关 键 词：软组织肿瘤 血管瘤样纤维组织细胞瘤 诊断 鉴别诊断 免疫组化 分子病理学 

分 类 号：R738.6[医药卫生—肿瘤]