儿童指状突树突细胞肉瘤1例并文献复习

摘要

目的：探讨指状突树突细胞肉瘤（interdigitating dendritic cell sarcoma，IDCS ）的临床特点、病理学特征、鉴别诊断、治疗及预后。方法报道1例发生于腹股沟淋巴结内的幼儿IDCS 的临床资料，并在Pubmed和国内CNKI、万方等医学文献数据库，采用指状突树突细胞肉瘤、儿童、病例报道为关键词进行检索，对其中5篇文献报道的8例儿童IDCS进行文献复习。结果9例指状突树突细胞肉瘤患儿：年龄8月-15岁，男4例，女5例；首发症状各异，均为淋巴结活检术后病理诊断，免疫组织化学显示瘤细胞阳性表达CD68、S-100，CD1α、CD35、CD20、CD3、CD23、MPO均阴性。结论指状突树突细胞肉瘤是一种罕见的恶性肿瘤。病理学免疫表型是诊断本病的重要依据，完整切除肿瘤可能是治愈IDCS的有效手段。%Objective Toexploretheclinicalmanifestation,pathologicalfindings,differential diagnosis,therapyandprognosisofinterdigitatingdendriticcellsarcoma(IDCS).Methods Theclinical data of one case with IDCS was reviewed and the 8 cases searched in Pubmed,CNKI and Wanfang database in the past 1 0 years ,using the interdigitating dendritic cell sarcoma,children,case report as keyword, werereviewedandanalyzed.Results All9casesofIDCSwerediagnosedbasedonpathology.Tumorcells expressed S1 00, CD68. CD4, CD1α, CD35, CD20, CD3, CD23 and MPO were all negative. Conclusions IDCSisakindofunusualmalignanttumor.Immuohistochemicalmethodsplaysakeyrolein diagnosis.Complete removal of the tumor my be the optional treatment.