首发于头颈部的Rosai-Dorfman病临床分析

目的探讨首发于头颈部的Rosai Dorfman病（Rosai Dorfman disease，RDD）临床表现、治疗方法及预后。方法回顾性分析1986~2017年收治的首发于头颈部的RDD患者10例，分析其一般情况、临床表现、病理特征、治疗经过及预后。结果10例首发于头颈部的RDD患者中男6例，女4例，确诊RDD时中位数年龄为36.5岁（22~77岁）。病变累及最多的部位为鼻部（8例）、喉部（6例），其中8例为≥2个部位受累。RDD临床表现与病变累及的部位及范围有关。治疗多采用激素+手术切除。中位数随访时间89个月（3~384个月），1例失访，1例治愈，其余8例带病生存。结论确诊RDD的患者需进行耳鼻咽喉科详尽的检查。患者的临床表现及影像学检查缺乏特异性。病理及免疫组织化学检查可确诊。采用激素+手术治疗的方式患者全身情况及头颈部病变可基本稳定，但仍需长期随诊。一般预后良好.

Clinical analysis of Rosai Dorfman disease in head and neck

ObjectiveTo analyze the clinical manifestations, treatment method, and prognosis of Rosai Dorfman disease (RDD) in head and neck.MethodsClinical data of 10 cases suffering from RDD in head and neck region between 1986 and 2017 were analyzed retrospectively. The data analyzed included clinical and pathological features, treatment and prognosis.ResultsOf the 10 patients, 6 were male and 4 were female. Their age ranged from 22 to 27 years old with a median age of 36.5. The lesions mostly involved nose (9 cases) and throat (6 cases). Eight cases had 2 or more regions involved. The clinical features were related to the locations and extent of the lesions. Glucocorticoid treatment combined with surgical resection was carried out for these patients. The median follow up duration was 90 months (3 to 384 months). One case was lost to follow up, one case was cured, and the remaining 8 cases were alive with disease.ConclusionsAll the patients diagnosed as RDD need a thorough examination of otolaryngology. The clinical characteristics and imaging features of RDD are nonspecific. The diagnosis for RDD relies on histopathologic examination of biopsy. The regimen of glucocorticoid therapy combined with surgical resection can stabilize the patients'' condition with favourable prognosis. Regular follow up is important in the management of this disease.