摘要Rosai-Dorfman病（RDD）是一种良性自限性疾病，其主要特征是淋巴结肿大和组织细胞吞噬大量淋巴细胞。本文报道1例颅内外非沟通性RDD，患者因“发现左顶部头皮肿物4个月，右下肢麻木半个月余”于2020年5月入住十堰市太和医院。患者行头颅磁共振成像（MRI）平扫+增强检查示：左顶骨病灶呈轻度不均匀强化，其内外侧软组织呈明显强化，局部内外侧软组织明显不规则增厚，以外侧为著，大小约3.2 cm×4.7 cm，邻近脑实质受压。行左顶部肿物及颅内肿物切除术后，病理结果显示梭形细胞增生伴炎性反应，免疫组织化学染色结果支持RDD诊断。患者术后7个月神经功能基本恢复正常，头颅MRI检查也未见病变复发，治疗效果满意。

abstractsRosai-Dorfman disease (RDD) is a benign self-limited disease characterized by lymphadenopathy and phagocytosis of lymphocytes by histiocytes. A case of intracranial-extracranial non communicating RDD was reported in this paper. The patient was admitted to Shiyan Taihe Hospital in May 2020 because of "the left top scalp tumor was found for 4 months, and the right lower limb was numb for more than half a month". The plain scan and enhanced scan of the patient′s head magnetic resonance imaging (MRI) showed that the disease focus of the left parietal bone was slightly uneven enhanced, its internal and external soft tissues were significantly enhanced, and the local internal and external soft tissues were significantly thickened irregularly, with the size of about 3.2 cm× 4.7 cm, and adjacent brain parenchyma was compressed. After resection of left top mass and intracranial mass, pathological results showed spindle cell proliferation with inflammatory reaction, and immunohistochemical staining results supported the diagnosis of RDD. The neurological function of the patient recovered to normal basically 7 months after operation, and no recurrence of the disease was found in the MRI examination of the head. The treatment effect was satisfactory.