Molina-Carrión Luis Enrique, Mendoza-Álvarez Sergio Alberto, Vera-Lastra Olga Lidia, Caldera-Duarte Agustín, Lara-Torres Héctor, Hernández-González Claudia
Servicio de Neurología, Hospital de Especialidades, Centro Médico Nacional La Raza, Instituto Mexicano del Seguro Social, Distrito Federal, México.
Rev Med Inst Mex Seguro Soc. 2014 Mar-Apr;52(2):218-23.
Rosai-Dorfman disease, known as well as sinus histiocytosis with massive lymphadenopathy, is a histiocytic proliferative disorder which may affect, with an extranodal presentation, the central nervous system, in 5 % of cases with exceptional reports of simultaneous development of spinal and cranial tumors. When it affects the central nervous system it appears more in men and it is shown as a mass in the cranial dura mater or in the spinal cord. The clinical symptoms of Rosai-Dorfman disease are fever, general malayse, weight loss, and nocturnal diaphoresis. Also, when Rosai-Dorfman disease affects the spinal cord, it has an impact on the thoracic spine, which causes paraparesis, quadriparesis, and sensory disorder. Histopathologically, the lymph nodes show emperipolesis. The diagnosis of Rosai-Dorfman disease is usually good, since 40 % of the patients present a spontaneous remission if they are treated with oral corticosteroids, even though the lesion can be managed with fractionated radiotherapy or with radical surgery. We report the case of a 34-year-old male who started with spinal injuries, and a year later showed intracranial lesions.
罗萨伊-多夫曼病,又称伴有巨大淋巴结病的窦性组织细胞增多症,是一种组织细胞增生性疾病,在5%的病例中可能以结外表现累及中枢神经系统,有同时发生脊柱和颅骨肿瘤的罕见报道。当它累及中枢神经系统时,男性更为多见,表现为硬脑膜或脊髓内的肿块。罗萨伊-多夫曼病的临床症状有发热、全身不适、体重减轻和夜间盗汗。此外,当罗萨伊-多夫曼病累及脊髓时,会影响胸椎,导致双下肢轻瘫、四肢瘫和感觉障碍。组织病理学上,淋巴结显示有血细胞吞噬现象。罗萨伊-多夫曼病的诊断通常较好,因为40%的患者口服皮质类固醇治疗后会出现自发缓解,尽管病变也可用分次放疗或根治性手术治疗。我们报告一例34岁男性患者,最初出现脊柱损伤,一年后出现颅内病变。