乳腺Rosai-Dorfman病12例临床病理特征分析

Clinicopathologic analysis of extranodal Rosai-Dorfman disease of breast： a report of 12 cases

目的探讨乳腺Rosai－Dorfman病（Rosai－Dorfman disease, RDD）的病理形态学、诊断及鉴别诊断。方法收集12例乳腺RDD，进行HE染色和免疫组织化学检测，光镜下观察组织学特征及免疫表型，并结合文献进行分析和讨论。结果12例乳腺RDD患者均为女性，年龄15～55岁，平均37岁，其中右乳7例，左乳5例。1例伴有腋窝淋巴结肿大，其余均为乳腺单发病灶。肿物呈不规则结节状，主要位于乳腺实质内，可累及周围脂肪组织。镜下淋巴细胞、组织细胞弥漫增生伴大量浆细胞浸润，再现淋巴结中的淋巴滤泡及扩张的＂窦＂，形成明暗相间的组织学特征。增生的组织细胞呈梭形或多边形，排列成模糊的席纹状，可有一定程度异型性，间质纤维化及胶原化明显，可见较多的泡沫细胞。特征性窦性组织细胞及淋巴细胞伸入现象较为少见或不明显。免疫组织化学示组织细胞S－100蛋白、CD68、CD163阳性，CD1a阴性。结论乳腺RDD临床上应与各种炎性病变、朗格汉斯细胞组织细胞增生症、Erdheim－Chester病、IgG4相关硬化性疾病、纤维组织细胞瘤、炎症型恶性纤维组织细胞瘤、霍奇金淋巴瘤等鉴别。低倍镜下明暗相间的组织学特征是诊断乳腺RDD的重要线索，特征性的淋巴细胞伸入现象具有诊断意义，免疫组织化学S－100蛋白、CD68、CD163阳性有助于诊断。

Objective To investigate the clinicopathologic features, diagnosis and differential diagnosis of extranodal Rosai-Dorfman disease （RDD） of the breast. Methods Twelve cases of extranodal RDD of the breast were analyzed using hematoxylin-eosin stain and immunohistochemical staining. The morphological features and immunophenotype were observed by light microscopy, accompanied with a review of the literature. Results Twelve cases of extranodal RDD of the breast are presented. The patients were women and their ages ranged from 15 to 55 years （mean = 37 years）. Except one patient with involvement of the right breast and ipsilateral axillary lymph nodes, other eleven patients＇ disease confined to the breast. Seven cases located in the right breast, the other five cases in the left breast. Microscopically, it showed a typical morphology, characterized by diffuse infiltration of large histiocytes, lymphocytes and plasmocytes that formed irregular nodular structure with light and dark appearance under microscope. Spindling of histiocytosis often arranged in a vague storiform with prominent fibrosis, accumulation of foamy histiocytes and scattered atypical nuclei. The characteristic histocytes and emperipolesis could not be seen obviously in extranodal RDD of the breast. Immunohistochemical staining showed histocytes were strongly positive for S-100 protein, CD68 and CD163, while negative for CDIa. Conclusions Extranodal RDD of the breast is very rare. Because of unconspicuous emperipolesis and lack of specificity in clinic and images, extranodal RDD of the breast is easy to be misdiagnosed as other histiocytosis. The microscopic differential diagnosis includes idiopathic granulomatous mastitis, infective granulomas, langerhans cell histiocytosis, IgG4 related sclerosing disease, Erdheim-Chester disease, benign fibrous histiocytoma, malignant fibrous histiocytoma and Hodgkin Lymphoma. Light and dark appearance under microscope is the important clue in diagnosis of extranodal RDD in breast. Immunohistochemistry is also helpful.