血管瘤样纤维组织细胞瘤14例临床病理学观察  

Angiomatoid fibrous histiocytoma:a clinicopathologic study of fourteen cases

作　　者：杨宝凤[1] 伏利兵[1] 张楠[1] 姚兴凤[1] 徐佳童 管晓星 王建文 何乐健[1] YANG Baofeng;FU Libing;ZHANG Nan;YAO Xingfeng;XU Jiatong;GUAN Xiaoxing;WANG Jianwen;HE Lejian(Department of Pathology,Beijing Children’s Hospital,Capital Medical University,Beijing 100045,China)

机构地区：[1]首都医科大学附属北京儿童医院病理科,北京100045

出　　处：《临床与实验病理学杂志》2024年第8期818-823,共6页Chinese Journal of Clinical and Experimental Pathology

摘　　要：目的探讨血管瘤样纤维组织细胞瘤(angiomatoid fibrous histiocytoma,AFH)的临床病理特征及分子学改变,分析其诊断、鉴别诊断及预后。方法收集14例AFH的临床、病理及随访资料,并文献复习。结果14例AFH患儿中,男童11例,女童3例;年龄11个月~12岁11个月,平均5.9岁。肿瘤位于四肢3例,躯干5例,头颈部5例,颅内1例。镜下肿瘤细胞核呈空泡状,合体样、漩涡状排列,可见纤维性假包膜及淋巴细胞鞘。9例可见假血管腔隙,2例可见钙化,2例核分裂活跃(活跃处11个/10 HPF)。3例镜下见硬化、黏液样间质。免疫表型:desmin(10/14)、EMA(12/14)、CD99(12/14)、SMA(9/12)、ALK(7/8)阳性,Ki67平均增殖指数16%。分子检测EWSR1基因断裂7例、EWSR1-ATF1融合2例、EWSR1-CREB1融合2例。14例患儿平均随访46个月,均无复发、转移。结论AFH是一种交界性或低度恶性肿瘤,儿童患者预后良好,很少复发或转移。诊断及鉴别诊断需要结合临床特点、组织形态、免疫组化及EWSR1、FUS基因检测综合分析。Purpose To study the clinical and pathological features of angiomatoid fibrous histocytoma(AFH)and to explore its diagnosis,differential diagnosis and prognosis.Methods The clinicopathological and follow-up data were analyzed in 14 cases of AFH,and the literatures were reviewed.Results There were 11 males and 3 females.The age ranged from 11 months to 12 years and 11 months,with average 5.9 years.3 cases were located in limbs,and 5 cases in trunk,5 cases in head and neck region,and 1 of intracranial tumor.Histologically,14 cases were composed of fibrous capsules and lymphocyte sheaths,and cell nucleus were vacuolar,forming fascicles with focal whirling and synteny.Intralesional pseudoangiomatous spaces were noted in 9 cases.Calcification was found in 2 cases.2 cases showed high mitotic acticity(11/10 HPF).Sclerosing and/or myxoid stroma was seen in 3 cases.Tumors were immunopositive for desmin(10/14),EMA(12/14),CD99(12/14),SMA(9/12),ALK(7/8),and the average of Ki67 index was 16%.7 cases harbored EWSR1 rearrangenent(partner gene not identified),2 cases had EWSR1-ATF1 fusion and 2 EWSR1-CREB1 fusion.Clinical follow-up information was available for 14 cases(average 46 months).All the 14 cases were alive without recurrence and metastasis.Conclusion AFH is a borderline or low-grade malignant tumor,often demonstrates indolent behavior in children,but rarely recurs and metastasizes.The diagnosis and differential diagnosis require a comprehensive analysis of clinical features,histopathologic changes,immunohistochemical finding and EWSR1 or FUS gene detection results.

关 键 词：软组织肿瘤 血管瘤样纤维组织细胞瘤 诊断 

分 类 号：R738.6[医药卫生—肿瘤]