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目的报道1例S-100(+)的腹膜后炎症性恶性纤维组织细胞瘤并探讨其与Rosai-Dorfman病的鉴别。方法进行临床病理和免疫组化分析,结合文献讨论其诊断与鉴别。结果患者女性,88岁。腹痛1个月。CT检查示腹膜后巨大肿块,广泛浸润邻近器官。病理检查示肿瘤由不同分化程度的组织细胞、泡沫细胞、纤维细胞及多种炎性细胞构成。肿瘤性组织细胞及纤维母细胞形成明显的车辐状结构,组织细胞无吞噬淋巴细胞。免疫组化:组织细胞S-100、CD68和vimentin(+);而EMA、CD21、CD35、ALK-1、CD1α和desmin(-)。结论炎症性恶性纤维组织细胞瘤表达S-100蛋白相当罕见,与Rosai-Dorfman病鉴别诊断需要结合临床特点、组织病理学和免疫组化综合分析。形态学上炎症性恶性纤维组织细胞瘤中的组织细胞比Rosai-Dorfman病中的组织细胞小、形态多样、中度异型性、出现病理性核分裂象,无吞噬淋巴细胞现象和背景中有多量中性粒细胞是与Rosai-Dorfman病鉴别诊断的要点。
Objective To report a case of retroperitoneal inflammatory malignant fibrous histiocytoma of S-100 (+) and to explore its relationship with Rosai-Dorfman’s disease. Methods Clinicopathological and immunohistochemical analysis, combined with the literature to discuss its diagnosis and identification. Results Female patient, 88 years old. Abdominal pain for 1 month. CT examination showed a large retroperitoneal mass, extensive infiltration of neighboring organs. Pathological examination revealed tumors of different degrees of differentiation of tissue cells, foam cells, fibroblasts and a variety of inflammatory cells. Tumor cells and fibroblasts formed a pronounced car-like structure, cells without phagocytic lymphocytes. Immunohistochemistry: Tissue cells S-100, CD68 and vimentin (+); while EMA, CD21, CD35, ALK-1, CD1a and desmin (-). Conclusion The expression of S-100 protein in inflammatory malignant fibrous histiocytoma is quite rare. The differential diagnosis with Rosai-Dorfman disease needs to be combined with clinical features, histopathology and immunohistochemical analysis. Morphologically, histological cells in inflammatory malignant fibrous histiocytomas are smaller and more diverse than those in Rosai-Dorfman’s disease with moderate atypia, with pathological mitosis, no phagocytic lymphocytosis, and a significant amount of background Glioblastoma is a differential diagnosis of Rosai-Dorfman disease.