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肝脏滤泡性树突状细胞肿瘤:两例病例的临床病理及爱泼斯坦-巴尔病毒研究

Follicular dendritic cell tumor of the liver: a clinicopathologic and Epstein-Barr virus study of two cases.

作者信息

Chen T C, Kuo T T, Ng K F

机构信息

Department of Pathology, Chang Gung Memorial Hospital, Chang Gung University School of Medicine, Kwei San, Tao Yuan, Taiwan.

出版信息

Mod Pathol. 2001 Apr;14(4):354-60. doi: 10.1038/modpathol.3880315.

Abstract

Two cases of hepatic follicular dendritic cell (FDC) tumor are described. Both patients were female, aged 57 and 51 years. They presented with epigastralgia or abdominal fullness and weight loss. The first patient refused surgical resection. She developed progressive polyclonal gammopathy and then bilateral purpura over the legs. Skin biopsy revealed leukocytoclastic vasculitis with granular vascular deposits of IgA and C3. The second patient had marked peripheral blood and tissue eosinophilia. The histological diagnosis was confirmed by positive staining for CD21 and CD23. The stromal lymphocytes were predominantly composed of CD3(+)and CD8(+) cells. In situ hybridization for EBER showed a positive nuclear signal in tumor cells but not in inflammatory cells. Polymerase chain reaction amplification for Exon 3 of the latent membrane protein-1 (LMP-1) gene showed a characteristic 30-bp deletion between nucleotides 168282 and 168253, corresponding to the B95-8 sequence. The unique clinicopathological features of our cases have not been reported for FDC tumors before. The clinical significance of the 30-bp deletion in Exon 3 of the LMP-1 gene in FDC tumor of the liver warrants further investigation.

摘要

本文描述了两例肝滤泡树突状细胞(FDC)肿瘤。两名患者均为女性,年龄分别为57岁和51岁。她们表现为上腹部疼痛或腹胀以及体重减轻。第一名患者拒绝手术切除。她出现了进行性多克隆丙种球蛋白病,随后双下肢出现紫癜。皮肤活检显示白细胞破碎性血管炎,伴有IgA和C3的颗粒状血管沉积。第二名患者外周血和组织中嗜酸性粒细胞明显增多。通过CD21和CD23阳性染色确诊组织学诊断。基质淋巴细胞主要由CD3(+)和CD8(+)细胞组成。EBER原位杂交显示肿瘤细胞中有阳性核信号,而炎症细胞中没有。潜伏膜蛋白-1(LMP-1)基因外显子3的聚合酶链反应扩增显示,在核苷酸168282和168253之间有一个30bp的特征性缺失,与B95-8序列相对应。我们病例独特的临床病理特征此前尚未见FDC肿瘤的报道。肝FDC肿瘤中LMP-1基因外显子3中30bp缺失的临床意义值得进一步研究。

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