指突状树突细胞肉瘤临床研究进展

The clinical advances of interdigitating dendritic cell sarcoma

指突状树突细胞肉瘤(IDCS)是一种来源于指突状树突细胞(IDC)的罕见的恶性造血组织肿瘤,是树突细胞瘤(DCN)的一种。目前对于IDCS的诊断主要依靠临床表现、细胞形态学及免疫组化相关的特点。因为其极为罕见,还没有关于它的前瞻性研究,而且对其明确的治疗方案及预后评估尚未形成统一标准。为了更好的了解这个疾病的生物学、诊断、鉴别诊断、治疗及预后等相关指标,本文查阅了1974至2016年各国的病例报告及相关文献,对其作一概述。

Interdigitating dendritic cell sarcoma（IDCS） is a rare malignant hematopoietic tumor derived from dendritic dendritic cells（IDC） and is a type of dendritic cell neoplasms （DCN）.At present,the diagnosis of IDCS relies mainly on the clinical manifestations,cell morphology and immunohistochemical characteristics.Because it is extremely rare,there is no prospective study on it,and its clear treatment plan and prognosis assessment has not yet formed a unified standard.In order to get a better understanding of the biology,diagnosis,differential diagnosis,treatment and prognosis of this disease,this paper reviews the case reports and related literatures from 1974 to 2016,and summarizes it.