Abstract：
Rosai-Dorfman disease (RDD) is a non-Langerhans cell histiocytosis which usually presents as painless lymphadenopathy. Extranodal involvement is known to occur in various organs, and less than ten cases with primary pancreatic involvement have been reported previously. This case report details the clinical course of an elderly female, presenting with upper abdominal discomfort and imaging suggestive of malignancy. Multiple non-diagnostic fine-needle aspirations were followed by surgical intervention. Histopathological evaluation revealed a pancreatic mass with characteristic features of RDD. The large hallmark RDD histiocytes showed pale, watery-clear cytoplasm, central round nucleus, and prominent nucleolus, with and without lymphocyte emperipolesis. The RDD histiocytes showed positive immunostaining for CD68, CD163, S100 (nuclear and cytoplasmic), OCT-2, Cyclin D1 and are negative for CD1a, Factor XIIIa, fascin and langerin. This case underscores the importance of considering RDD in the differential diagnosis of pancreatic masses alongwith comprehensive evaluation, multidisciplinary approach and pancreatic core needle biopsy evaluation.
摘要：
Rosai-Dorfman病（RDD）是非朗格汉斯细胞组织细胞增生症，通常表现为无痛性淋巴结病。已知结外受累发生在各种器官中，以前报道的原发性胰腺受累病例不到10例。此病例报告详细介绍了一名老年女性的临床过程，表现为上腹部不适，影像学提示恶性肿瘤。多次非诊断性细针穿刺后进行手术干预。组织病理学评估显示胰腺肿块具有RDD的特征性特征。大标志的RDD组织细胞显示苍白，水样透明的细胞质，中央圆形核，和突出的核仁，有和没有淋巴细胞外溢。RDD组织细胞对CD68，CD163，S100（细胞核和细胞质）显示阳性免疫染色，OCT-2，细胞周期蛋白D1，CD1a阴性，因子XIIIa，Fascin和Langerin.该病例强调了在综合评估的基础上，在胰腺肿块的鉴别诊断中考虑RDD的重要性。多学科方法和胰腺芯针活检评估。
