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指状突树突状细胞肉瘤。4例报告并文献复习

Interdigitating dendritic cell sarcoma. A report of four cases and review of the literature.

作者信息

Gaertner E M, Tsokos M, Derringer G A, Neuhauser T S, Arciero C, Andriko J A

机构信息

Dept of Dermatopathology, 14th St and Alaska Ave NW, Bldg 54, Washington, DC 20307, USA.

出版信息

Am J Clin Pathol. 2001 Apr;115(4):589-97. doi: 10.1309/M95G-7DQ2-TLQL-7Q11.

DOI:10.1309/M95G-7DQ2-TLQL-7Q11
PMID:11293908
Abstract

To better define the clinical and pathologic features of interdigitating dendritic cell sarcoma (IDCS), we report 4 cases, including the first reported in the tonsil. There were 2 male and 2 female patients (mean age, 70 years). Sites of tumor included 1 case each in the right cervical lymph node, left axillary lymph node, right tonsil, and right inguinal lymph node. Histologically, all showed diffuse effacement of the lymphoid tissue by pleomorphic round to spindled cells with convoluted nuclei and abundant eosinophilic cytoplasm. All were immunoreactive for S-100, CD68, lysozyme, and vimentin. CD45 was positive in 3 cases and CD1a in 1 case. Fascin was positive in 3 cases. Other immunostains, including CD3, CD20, CD21, CD30, actin, cytokeratin, and HMB-45, were negative. Ultrastructurally, the tumor cells were elongated and showed indented nuclei, variable numbers of lysosomes, and interdigitating cytoplasmic processes. Follow-up was available for all cases. One patient died of widespread disease 2 months after diagnosis. One was alive with metastatic lung disease at 12 months. Two patients were disease free at 5 and 9 months.

摘要

为了更好地界定指突状树突细胞肉瘤(IDCS)的临床和病理特征,我们报告4例病例,包括首例报道的扁桃体IDCS。患者2男2女(平均年龄70岁)。肿瘤部位包括右颈淋巴结、左腋窝淋巴结、右扁桃体和右腹股沟淋巴结各1例。组织学上,所有病例均显示淋巴组织被多形性圆形至梭形细胞弥漫性取代,细胞核呈卷曲状,嗜酸性细胞质丰富。所有病例S-100、CD68、溶菌酶和波形蛋白免疫反应均呈阳性。3例CD45呈阳性,1例CD1a呈阳性。3例fascin呈阳性。其他免疫组化染色,包括CD3、CD20、CD21、CD30、肌动蛋白、细胞角蛋白和HMB-45均为阴性。超微结构上,肿瘤细胞呈细长形,细胞核呈凹陷状,有数量不等的溶酶体和指突状细胞质突起。所有病例均获得随访。1例患者诊断后2个月死于广泛转移。1例患者在12个月时存活但有肺转移。2例患者分别在5个月和9个月时无疾病复发。

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