| | | 软组织Rosai
| | 药学见习论文 作者：杨兆瑞，路光中，胡宏慧，张宜玲
【关键词】 组织
RosaiDorfman disease of soft tissue: A case report and literature review
【Abstract】 AIM: To study the pathological diagnosis, immunohistochemical features and differential diagnosis of RosaiDorfman disease (RDD) of soft tissue. METHODS: A case of RDD of soft tissue on the lower limb was investigated by light microscopy, HE stain, silver stain, Masson stain and immunohistochemical stains for S100, KP1, MAC387, VIM, UCHL1, L26 and CD34, and related literatures were reviewed. RESULTS: Microscopically, the lesion consisted of abundant collagen fibrils, proliferated histiocytes and numerous infiltrated plasmocytes, lymphocytes and eosinophilic granulocytes. Fusiform or polygonal histiocytes had abundant, granular, eosinophilic cytoplasm or clear cytoplasm containing radially oriented strands or resembled xanthoma cells. Emperipolesis of numerous small lymphocytes was seen in the cytoplasm of some large histiocytes. Proliferated collagen fibrils and fusiform histiocytes formed atypical storiform pattern. Immunohistochemically, the proliferated histiocytes were positive for S100, KP1, MAC387 and VIM. Masson stain and silver stain revealed the abundant collagen fibrils in the lesion. The histiocytes were negative for PASAB. CONCLUSION: RDD of soft tissue is rare and liable to be misdiagnosed in pathologic diagnosis. The histopathologic features are therefore of significance in differential diagnosis.
【Keywords】 RosaiDorfman disease；soft tissue；histiocyte；emperipolesis
【摘要】 目的药学见习论文：探讨软组织罗道病（RDD）的药学见习论文病理诊断和免疫组化特点以及鉴别诊断. 方法：通过HE、免疫组化及特殊染色观察1例下肢软组织RDD并复习文献. 结果： 镜下可见丰富的胶原纤维和组织细胞增生以及大量浆细胞、淋巴细胞和嗜酸粒细胞浸润，组织细胞呈梭形、多边形，胞质嗜酸性颗粒状、放射条纹状或泡沫状，一些大组织细胞质内有多量小淋巴细胞. 增生的胶原和梭形组织细胞构成不典型Storiform结构；组织细胞表达S100，KP1，MAC387，VIM，Masson三色及网状纤维染色显示大量胶原纤维增生，组织细胞PASAB染色阴性. 结论： 软组织RDD是临床罕见的疾病，在病理诊断方面，易误诊为其他疾病，明确病理组织学特点在鉴别诊断中有重要意义.
【关键词】 RosaiDorfman病；软组织；组织细胞；伸入运动
0引言
罗道病（RosaiDorfman disease, RDD）也称窦组织细胞增生伴巨大淋巴结病（sinus histiocytosis with massive lymphadenopathy，SHML），是一种十分少见的淋巴结或结外的反应性病变. 开始报道RDD发生于淋巴结［1］，以后报道见于任何器官系统［2-8］. 国外有关淋巴结外RDD的文献不多，国内仅有少数几篇［7-8］. 我们于2003年诊断1例软组织RDD，现结合文献复习，对其临床病理特征、免疫表型、鉴别诊断及临床治疗方法和疗效报道如下.
1对象和方法
1.1对象女性， 61岁，因左大腿肿块近1 a，右臀部肿块切除术后复发0.5 a于20030106入院. 自述1 a前发现左大腿及右臀部肿块，因左大腿肿块小未引起注意. 200202在当地医院行“右臀部肿块切除术”，病理诊断为“软组织淋巴细胞瘤样增生”. 术后0.5 a，右臀部切口疤痕处又发现2个小结节；左大腿外侧肿块逐渐增大伴有酸胀感. 有Ⅱ型糖尿病史10 a余，口服降糖药控制，效果稳定. 腹股沟及其他部位浅表淋巴结未触及肿大，肝、脾无肿大. 实验室检查：血沉28 mm/h，血蛋白电泳、风湿及类风湿因子检查结果无异常. 术后随访2 a，肿块无复发.
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