淋巴结外Rosai-Dorfman病3例临床病理观察

Extranodal Rosai-Dorfman disease: a clinicopathological analysis of three cases

目的探讨原发性淋巴结外Rosai-Dorfman病（RDD）的临床病理学特征、诊断及鉴别诊断。方法分析3例结外Rosai-Dorfman病的临床特点,并行HE和免疫组化染色。结果 3例中男性1例,女性2例,年龄40~72岁,平均57岁。病变分别位于右鼻腔、左大腿皮下软组织及子宫内膜。组织学特点为组织细胞明显增生,细胞体积大,胞质丰富淡染,与间质内淋巴细胞和浆细胞形成（淡染、深染相间）类似淋巴窦隙样的结构,组织细胞胞质内可见吞噬的淋巴细胞、浆细胞。免疫组化示组织细胞S-100蛋白（＋）,部分CD163（＋）。结论结外RosaiDorfman病较为少见,有特征性的病理学特点,系统累及可能性较小,预后较好,有一定的局部复发率。组织学上应与Langerhans细胞组织细胞增生症等鉴别。

Objective To study the clinicopathological features, diagnosis and differential diagnosis of extranodal Rosai- Dorfman disease （RDD）. Methods Clinical feature, sections of hematoxylin-eosin and immunohistochemieal staining were analyzed in 3 cases of extranodal RDD. Results The patients were 1 males and 2 females, aged from 40 to 72 years（ mean 57 years ）. The lesions were located in the right nasal cavity subcutaneous soft tissue of left thigh and endometrium. Histologically, the lesions were characterized by variable numbers of large histiocytes with emperipolesis, accompanied by infiltration of lymphocytes and plasma cells. The histiocytes were positive for S-100 protein and CD163. Conclusion Extranodal RDD is a very rare histiocytic proliferative disorder which has distinct morphologic feature. Systemic involvement is rare and its prognosis good, but patients have some of recurrence rate. It should be differentiated from other diseases such as Langerhans cell histiocytosis.