CAJ | 학술논문

目的：探讨血管瘤样纤维组织细胞瘤的临床病理特征、分子遗传学改变、病理诊断及鉴别诊断。方法：收集2例血管瘤样纤维组织细胞瘤，复习相关文献，分析其临床病理学特征。采用免疫组织化学和荧光原位杂交方法分别观察其免疫表型和EWSR1基因重排情况。结果：2位患者年龄为9岁和24岁，分别表现为左上臂和颈前区包块。大体上，肿瘤界限清楚、直径1．5～2．0 cm。镜下，瘤组织周边见淋巴细胞、浆细胞浸润带，并被一层较厚的纤维性假包膜包绕；瘤细胞梭形和胖梭形，异型性轻到重度，可见多核巨细胞，核分裂像可见。其中1例瘤细胞巢内含有出血性囊腔，另一例为实体型。免疫组织化学显示瘤细胞均弥漫表达vimentin，部分细胞表达CD68、CD99，少量细胞表达SMA、EMA；Ki－67增殖指数为3％～10％；荧光原位杂交检测结果显示2例均存在EWSR1基因重排。2例均经手术完整切除肿块，随访54个月未见复发和转移。结论：血管瘤样纤维组织细胞瘤是少见的交界性肿瘤，多见于儿童和青少年，好发于四肢，其次位于躯干和头颈部，易与其他肿瘤混淆。熟悉这一病变的临床、病理形态特点，结合免疫组织化学和分子遗传学检测能够避免误诊。
목적：탐토혈관류양섬유조직세포류적림상병리특정、분자유전학개변、병리진단급감별진단。방법：수집2례혈관류양섬유조직세포류，복습상관문헌，분석기림상병이학특정。채용면역조직화학화형광원위잡교방법분별관찰기면역표형화EWSR1기인중배정황。결과：2위환자년령위9세화24세，분별표현위좌상비화경전구포괴。대체상，종류계한청초、직경1．5～2．0 cm。경하，류조직주변견림파세포、장세포침윤대，병피일층교후적섬유성가포막포요；류세포사형화반사형，이형성경도중도，가견다핵거세포，핵분렬상가견。기중1례류세포소내함유출혈성낭강，령일례위실체형。면역조직화학현시류세포균미만표체vimentin，부분세포표체CD68、CD99，소량세포표체SMA、EMA；Ki－67증식지수위3％～10％；형광원위잡교검측결과현시2례균존재EWSR1기인중배。2례균경수술완정절제종괴，수방54개월미견복발화전이。결론：혈관류양섬유조직세포류시소견적교계성종류，다견우인동화청소년，호발우사지，기차위우구간화두경부，역여기타종류혼효。숙실저일병변적림상、병리형태특점，결합면역조직화학화분자유전학검측능구피면오진。
Objective:To understand the clinicopathological and genetic features of angiomatoid fibrous histiocytoma ( AFH) for addressing the diagnosis and differential diagnosis of this entity.Methods:Clinicopahtological pictures of AFH were examined in 2 cases by histopathology, immunohistochemistry and fluorescence in situ hybridization (FISH), and the related literatures were reviewed.Results:The patients aged 9 and 24 years, and presented with a mass at elbow and neck, respectively.Both patients underwent simple excision.Gross examination showed a single mass with a clear boundary, measur-ing 1.5-2.0 cm in diameter.Microscopically, the distinctive histopathology were:①aggregated nodules of spindle or histocytoid cells, one with signifi-cant atypia, and the mitotic figures were all below two per 10 HPF; ②peritumoral chronic inflammatory cells infiltration; ③tumor cells surrounded by a dense fibrous pseudocapsule; and ④blood-filled cystic spaces being seen in one cases.The margins were negative in both cases.Immunohistochemical staining showed that tumor cells were positive for vimentin, focally positive for CD68, CD99, rarely positive for SMA and EMA.MyoD1, Myogenin, Myo-globin, CD21, CD23, CD35, CD34, ALK-1, S-100 protein, HMB45 and CKpan were all negative.Proliferation index was 3%-10%.EWSR1 rear-rangement was detected in both cases by FISH.Follow-up in 54 months showed no evidence of recurrence.Conclusions:AFH is an uncommon tumor and its correct diagnosis relies on clinical features and pathological examination .Immunohistochemical staining is useful for differential diagnosis , and EWSR1 rearrangement may be a potential biomarker for diagnosis .