首页|EBV阳性炎性滤泡树突状细胞肉瘤9例临床病理特征

EBV阳性炎性滤泡树突状细胞肉瘤9例临床病理特征

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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D240(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.
Clinicopathologic characteristics of Epstein-Barr virus-positive inflammatory follicular dendritic cell sarcoma:report of nine cases
Purpose To explore the clinicopathologic fea-tures of Epstein-Barr virus-positive inflammatory follicular den-dritic cell sarcoma(EBV+IFDCS).Methods The clinico-pathologic features of 9 cases of EBV+IFDCS were retrospective-ly 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.Fi-brinoid degeneration and hyaline degeneration in the vessel walls(6/9),eosinophilic infiltration(3/9),and epithelioid granulo-mas(2/9)were seen in some cases.The tumor cells expressed CD21(7/9),CD23(8/9)and CD35(9/9),partially ex-pressed 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 pa-tients 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 immunohisto-chemical detection play important roles in the diagnosis and dif-ferential diagnosis of EBV+IFDCS.Surgical resection is the main therapeutic intervention for EBV+IFDCS,and patients re-quire long-term post-surgical follow-up.

Epstein-Barr virus-positive inflammatory follicular dendritic cell sarcomaIgG4-related diseasesEBER in situ hy-bridizationprognosis

陈倩倩、罗雯雯、关结霞、梁琼

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中山大学附属第三医院病理科,广州 510000

EBV阳性炎性滤泡树突状细胞肉瘤 IgG4相关疾病 EBER原位杂交 预后

广州市科技计划项目

202102010141

2024

临床与实验病理学杂志
安徽医科大学,中华医学会安徽分会

临床与实验病理学杂志

CSTPCD北大核心
影响因子:0.776
ISSN:1001-7399
年,卷(期):2024.40(1)
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