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指突状树突细胞肉瘤:8例临床病理研究并文献复习

Interdigitating dendritic cell sarcoma: Clinicopathologic study of 8 cases with review of the literature.

作者信息

Xue Tian, Jiang Xiang-Nan, Wang Wei-Ge, Zhou Xiao-Yan, Li Xiao-Qiu

机构信息

Department of Pathology, Fudan University Shanghai Cancer Center, Shanghai, China; Department of Oncology, Shanghai Medical College, Fudan University, Shanghai, China.

Department of Pathology, Fudan University Shanghai Cancer Center, Shanghai, China; Department of Oncology, Shanghai Medical College, Fudan University, Shanghai, China.

出版信息

Ann Diagn Pathol. 2018 Jun;34:155-160. doi: 10.1016/j.anndiagpath.2018.03.008. Epub 2018 Mar 30.

DOI:10.1016/j.anndiagpath.2018.03.008
PMID:29660568
Abstract

To investigate the clinicopathologic features and differential diagnoses of interdigitating dendritic cell sarcoma (IDCS), the clinical, morphological and immunohistochemical features of eight cases of IDCS were collected and analyzed. Three patients were males and five were females, the mean age and the median age were 56.5 years and 57 years respectively. Clinically, the majority of cases involved lymph nodes. Microscopically, neoplastic cells were spindle or ovoid, forming fascicles or whorls. Every case had active mitosis figures. Immunohistochemically, these neoplastic cells were consistently positive for S100, but negative for CD21 and specific B-cell and T-cell associated antigens. Follow-up results were available in 7 cases, of which 5 cases of localized lesions survived, 2 cases died of organ involvement. Interdigitating dendritic cell sarcoma is an extremely rare neoplasm, with inferior prognosis and without standard treatment regimen. IDCS has similar but unique clinicopathologic features and the differential diagnoses include other histiocytic and dendritic cell neoplasms and malignant melanoma.

摘要

为探讨指状突树突状细胞肉瘤(IDCS)的临床病理特征及鉴别诊断,收集并分析8例IDCS的临床、形态学及免疫组化特征。3例为男性,5例为女性,平均年龄56.5岁,中位年龄57岁。临床上,大多数病例累及淋巴结。镜下,肿瘤细胞呈梭形或卵圆形,形成束状或漩涡状。每例均有活跃的有丝分裂象。免疫组化显示,这些肿瘤细胞S100始终呈阳性,但CD21及特异性B细胞和T细胞相关抗原呈阴性。7例有随访结果,其中5例局限性病变存活,2例死于器官受累。指状突树突状细胞肉瘤是一种极其罕见的肿瘤,预后较差且无标准治疗方案。IDCS具有相似但独特的临床病理特征,鉴别诊断包括其他组织细胞和树突状细胞肿瘤以及恶性黑色素瘤。

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