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脾脏EB病毒阳性炎症性滤泡树突状细胞肉瘤:一例报告及文献复习

Spleen EBV-positive inflammatory follicular dendritic cell sarcoma: a case report and literature review.

作者信息

Xiao Yi, Li Lanlan, Zhan Xiumei, Xu Juner, Chen Yewu, Zhao Qiuchan, Fu Yinghao, Luo Xian, Chen Huadi, Xu Hao

机构信息

Department of Hepatobiliary Surgery, Affiliated Hospital of Guangdong Medical University, Zhanjiang, Guangdong, China.

The First School of Clinical Medicine, Guangdong Medical University, Zhanjiang, Guangdong, China.

出版信息

Front Oncol. 2025 Aug 22;15:1640099. doi: 10.3389/fonc.2025.1640099. eCollection 2025.

Abstract

Epstein-Barr virus-positive inflammatory follicular dendritic cell sarcoma (EBV+ IFDCS) is a rare tumor that typically arises in the liver or spleen and is characterized by spindle-shaped cells within a dense lymphoplasmacytic background. We report a case of a 64-year-old woman with an incidental splenic mass found during routine imaging. MRI showed a 4.8 cm lesion with progressive enhancement. The patient underwent laparoscopic partial splenectomy. Histopathological examination revealed features consistent with EBV+ IFDCS, including positivity for CD21, CD23, CD35, SMA, EMA, and EBER. EBV+ IFDCS often presents with nonspecific symptoms and imaging findings, making diagnosis challenging. Definitive diagnosis relies on histology, immunohistochemistry, and confirmation of EBV infection. Most cases follow an indolent clinical course and have a favorable prognosis after complete surgical resection, though rare aggressive cases have been reported. Understanding its clinicopathological and molecular features is essential for accurate diagnosis and management.

摘要

爱泼斯坦-巴尔病毒阳性炎症性滤泡树突状细胞肉瘤(EBV+ IFDCS)是一种罕见肿瘤,通常发生于肝脏或脾脏,其特征为在密集的淋巴浆细胞背景中有梭形细胞。我们报告一例64岁女性,在常规影像学检查时偶然发现脾脏肿块。磁共振成像(MRI)显示一个4.8厘米的病灶,呈渐进性强化。患者接受了腹腔镜下部分脾切除术。组织病理学检查显示符合EBV+ IFDCS的特征,包括CD21、CD23、CD35、平滑肌肌动蛋白(SMA)、上皮膜抗原(EMA)和爱泼斯坦-巴尔病毒编码的小RNA(EBER)呈阳性。EBV+ IFDCS常表现为非特异性症状和影像学表现,诊断具有挑战性。明确诊断依赖于组织学、免疫组织化学以及EBV感染的确认。大多数病例临床过程呈惰性,完整手术切除后预后良好,不过也有罕见的侵袭性病例报道。了解其临床病理和分子特征对于准确诊断和治疗至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9b4c/12411919/dd50d43ff57c/fonc-15-1640099-g001.jpg

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