Triana-Pérez A B, Sánchez-Medina Y, Pérez-Del Rosario P A, Millán-Corada A M, Gómez-Perals L F, Domínguez-Báez J J
Servicio de Neurocirugía. Hospital Universitario Nuestra Señora de Candelaria. Santa Cruz de Tenerife.
Neurocirugia (Astur). 2011 Jun;22(3):255-60.
Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease (RDD) is an idiopathic histiocytosis that usually affects the lymph nodes. Occasionally it may affect the CNS, being exceptional intracranial involvement without lymph node lesions. In the absence of typical radiological signs, affected patients are generally operated under the suspicion of a meningioma. The histological diagnosis is obtained after the surgical procedure. It is a clinicopathological entity not well known, controversy exists about its pathogenesis, clinical course and therapeutic management.
We report the case of a 40-year-old male presented two generalized tonic-clonic seizures and brain MRI showed a left parieto-occipital extra-axial lesion extending into the posterior fossa, without presenting lesions at other levels. A partial resection of the lesion was performed and the histological findings were reported as Rosai-Dorfman disease.
Despite its low frequency, the ERD should be included in the differential diagnosis of dural-based masses, compared to more common, such as meningiomas. Due to lack of specificity of additional studies its diagnosis is fundamentally histologic. More research is needed to define the best therapeutic option.
伴巨大淋巴结病的窦性组织细胞增生症，也称为罗萨伊 - 多夫曼病（RDD），是一种特发性组织细胞增生症，通常累及淋巴结。偶尔它可能累及中枢神经系统，颅内受累而无淋巴结病变的情况较为罕见。在缺乏典型影像学征象的情况下，受影响的患者通常在怀疑为脑膜瘤的情况下接受手术。组织学诊断在手术后获得。这是一种临床病理实体，人们对其发病机制、临床过程和治疗管理存在争议。
我们报告了一例40岁男性，出现两次全身性强直 - 阵挛性发作，脑部MRI显示左侧顶枕部轴外病变延伸至后颅窝，其他层面未发现病变。对病变进行了部分切除，组织学检查结果报告为罗萨伊 - 多夫曼病。
尽管发病率较低，但与更常见的如脑膜瘤等相比，罗萨伊 - 多夫曼病应列入硬膜下肿块的鉴别诊断中。由于其他检查缺乏特异性，其诊断主要基于组织学。需要更多研究来确定最佳治疗方案。
