儿童颅内原发Rosai-Dorfman病1例及文献复习

颅内Rosai-Dorfman病(Rosai-Dorfman disease，RDD)是一类临床罕见的组织细胞增生性疾病。中南大学湘雅医院收治1例12岁颅内RDD患儿，主诉为头痛、头晕1个月，行肿瘤全切除手术，术后应用激素及化学药物治疗。随访8个月，病情稳定，未见肿瘤复发。对于此类肿瘤且有进展趋势者，应用立体定向活检术有助于明确诊断及手术方案的选择(如骨瓣取舍等)。本病以手术干预为主，辅以放射治疗、化学药物治疗及激素治疗等方案，且手术应在不影响神经功能的情况下力求全切肿瘤。

Intracranial Rosai-Dorfman disease in a child: A case report and literature review

Intracranial Rosai-Dorfman disease (RDD) is a rare clinical histiocytosis proliferative disease. A 12-year-old boy with dizziness and headache for 1 month was admitt ed into Pediatric Neurosurgeryof Xiangya Hospital, Central South University. Th e patient underwent total tumor resection and postoperative application of hormones and chemotherapy. During follow-up of 8 months, patient’scondition was stable and no tumor recurrence was observed. For patient with a trend of tumor progression, stereotactic biopsy can help to confirm the diagnosis and determine the surgical strategy such as disposal of bone flaps. The treatment is mainly based on surgical intervention, supplemented by radiotherapy, chemotherapy and hormone therapy. Without aff ecting the nerve function, the surgeon should try to completely resect the tumor.