EBV阳性炎性滤泡树突状细胞肉瘤9例临床病理特征

Clinicopathologic characteristics of Epstein-Barr virus-positive inflammatory follicular dendritic cell sarcoma:report of nine cases

目的探讨EBV阳性炎性滤泡树突状细胞肉瘤(epstein-barr virus-positive inflammatory follicular dendritic cell sarcoma,EBV^(+)IFDCS)的临床病理学特征。方法回顾性分析9例EBV^(+)IFDCS的临床病理特征、免疫表型及随访,行HE及免疫组化EnVision法检测,并复习相关文献。结果9例EBV^(+)IFDCS患者年龄22~78岁,平均44.7岁。7例发生于肝,2例发生于脾。肿瘤内可见血管壁纤维样及透明变性(6/9)、嗜酸性粒细胞浸润(3/9)和上皮样肉芽肿(2/9)。免疫表型:瘤细胞表达滤泡树突状细胞标志物CD21(7/9)、CD23(8/9)和CD35(9/9),部分表达SMA(6/9)和D2-40(1/9)。2例脾IgG4阳性浆细胞明显增高(80~135个/10 HPF),7例肝IgG4阳性浆细胞为0~36个/10 HPF。术后随访3~84个月,6例无瘤生存,2例转移,1例失访。结论EBV^(+)IFDCS罕见,低度恶性。EBER原位杂交和免疫组化检测在EBV^(+)IFDCS诊断和鉴别诊断中具有重要作用。治疗以手术切除为主,术后需长期随访。

Purpose To explore the clinicopathologic features of Epstein-Barr virus-positive inflammatory follicular dendritic cell sarcoma(EBV^(+)IFDCS).Methods The clinicopathologic features of 9 cases of EBV^(+)IFDCS were retrospectively analyzed and followed up.Results The age of 9 patients with EBV^(+)IFDCS ranged from 22 to 78 years(mean 44.7 years).7 cases occurred in the liver and 2 in the spleen.Fibrinoid degeneration and hyaline degeneration in the vessel walls(6/9),eosinophilic infiltration(3/9),and epithelioid granulomas(2/9)were seen in some cases.The tumor cells expressed CD21(7/9),CD23(8/9)and CD35(9/9),partially expressed SMA(6/9)and D2-40(1/9).It was noteworthy that 2 cases from the spleen accompanied by high expression of IgG4 plasma cells(80-135/10 HPF),and in the liver(0-36/10 HPF).All cases were followed up for 3-84 months,with 6 patients disease-free,2 patients underwent metastasis,1 patient lost of follow-up.Conclusion EBV^(+)IFDCS is a rare low-grade malignant tumor.EBER in situ hybridization and immunohistochemical detection play important roles in the diagnosis and differential diagnosis of EBV^(+)IFDCS.Surgical resection is the main therapeutic intervention for EBV^(+)IFDCS,and patients require long-term post-surgical follow-up.