脾脏炎性假瘤样滤泡树突细胞肉瘤2例及文献回顾

Inflammatory pseudotumor-like follicular dendritic cell sarcoma of the spleen:2 cases report and literature review

炎性假瘤样滤泡树突细胞肉瘤(inflammatory pseudotumor-like follicular dendritic cell sarcoma,IPT-like FDCS)是一种十分罕见的、被认为与EB病毒相关的恶性肿瘤。本文2例患者体检发现脾脏占位,完善检查后予腹腔镜下全脾脏切除术。病理示:炎性假瘤样滤泡树突细胞肉瘤,2例术后均未行放化疗。该疾病多无特征性临床表现,而影像学上则与肉瘤有重叠表现。镜下表现为炎症背景中可见较多的CD21、CD23和EBER阳性的梭形肿瘤细胞呈席纹状排列,由于肿瘤细胞与淋巴细胞交织分布,边界不清,故存在一定的诊断难度。本文报道这2例病例并结合文献复习,探讨其临床和病理学特点,以提高诊断认知。

Inflammatory pseudotumor-like follicular dendritic cell sarcoma(IPT-like FDCS)is a very rare malignant tumor that is considered to be associated with Epstein-Barr virus.Two patients in this report were generally healthy,and the spleen tumor was found during physical examination.After completing the examination,laparoscopic total splenectomy was performed,and the pathological result showed IPT-like FDCS.Postoperative chemoradiotherapy was not performed in either case.The disease has no characteristic clinical manifestations,and imaging overlaps with sarcoma.Microscopic manifestation showed CD21,CD23 and EBER positive spindle tumor cells in the inflammatory background with matted arrangement.Due to the interwoven distribution of tumor cells and lymphocytes,diagnosis is difficult.In this article,we report this two cases with literature review and summarize their clinical and pathological features to improve diagnostic cognition.