血管瘤样纤维组织细胞瘤的临床病理观察

摘要

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。%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 .