滤泡树突状细胞肉瘤(Follicular Dendritic Cell Sarcoma,FDCS)是一种罕见的起源于生发中心滤泡树突状细胞的恶性肿瘤,由于其临床表现、影像学表现和组织形态表现均无特异性,故往往被误诊为其他类型的肿瘤。本文从发病机制、病理学特点、诊断与治疗等多个方面对目前滤泡树突状细胞肉瘤的研究进展做一综述。 Follicular Dendritic Cell Sarcoma (FDCS) is a rare malignant tumor originated in the germinal center follicular dendritic cells. Because of its clinical manifestations, imaging findings and histological appearance are not specific , It is often misdiagnosed as other types of tumors. This article reviews the current research progress on the current follicular dendritic cell sarcoma from the pathogenesis, pathological features, diagnosis and treatment.