腹膜后炎症性恶性纤维组织细胞瘤与Rosai-Dorfman病的鉴别诊断  

Inflammatory malignant fibrohistiocytoma and its differential diagnosis with Rosai-Dorfman disease

作　　者：潘玉林[1] 谢蕴[1] 罗志谋[1] 金晓龙[2] 

机构地区：[1]上海市瑞金医院集团闵行医院病理科,上海201100 [2]上海交通大学医学院附属瑞金医院病理科,上海200025

出　　处：《诊断病理学杂志》2010年第1期42-45,共4页Chinese Journal of Diagnostic Pathology

摘　　要：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 study the histopathological characteristics of inflammatory malignant fibrohistocytoma with S- 100 expression and to discuss the differential diagnosis with other histiocytic proliferative diseases that are also positive for S-100 protein, especially for Rosai-Dorfman disease. Methods One case of inflammatory malignant fibrohistocytoma with S-100 positive was studied by light microscopy and immunohistochemistry. Result A 88-year old female presented abdominal pain for one month. The CT scan revealed a huge mass in retroperitoneal cavity, which was widely invaded local organs nearby. Pathologically, the tumor was mainly consisted of histiocytes, foamy cells, intermingled with fibroblasts, Touton giant cells, and variety of inflammatory cells. The neoplastic histiocytes and spindle fibroblasts arranged in a prominent storiform pattern. Tumor cells were positive for vimentin, S-100 and CD68, but negative for EMA, CD21, CD35, ALK-1, CD1α and desmin. Conclusion inflammatory malignant fibrohistocytoma expressing S-100 protein is very rare. To make differential diagnosis with Rosai-Dorfman disease, all clinical features, morphological characteristics and immunohistochemical phenotype should be taken into account. The smaller sizes of histiocytes in inflammatory malignant fibrohistiocytoma, moderate atypia and without emproloplosis are the key points to rule out the Rosai-dorfman disease.

关 键 词：炎症性纤维组织细胞瘤 Rosai—Dorfman病 临床病理特征 免疫组化 

分 类 号：R733.1[医药卫生—肿瘤]