Andriko J A, Morrison A, Colegial C H, Davis B J, Jones R V
Department of Hematopathology, Armed Forces Institute of Pathology, Washington, DC 20306, USA.
Mod Pathol. 2001 Mar;14(3):172-8. doi: 10.1038/modpathol.3880278.
Sinus histocytosis with massive lymphadenopathy, also known as Rosai-Dorfman Disease (RDD), is an idiopathic histiocytic proliferation affecting lymph nodes. Although extranodal involvement has been reported in diverse sites, central nervous system (CNS) manifestation, particularly in the absence of nodal disease is uncommon. We report 11 cases of RDD primary to the CNS without evidence of other sites of involvement. The cases included 7 males and 4 females ranging in age from 22 to 63 years (mean: 41 y). The patients presented with headaches, seizures, numbness, or paraplegia. Eight cases involved the cranial cavity and three cases, the spinal canal. Lesions were most often extra-axial and dura based. Only one presented in the CNS parenchyma. Histologically, the lesions consisted of variable numbers of pale-staining histocytes with emperipolesis often overshadowed by extensive lymphoplasmacytic infiltrates and fibrosis in the background. Special stains for organisms were negative. By immunohistochemical analysis, the characteristic histiocytes were positive for S100 protein and CD68 and negative for CD1a. Treatment consisted of surgical biopsy or excision. Follow-up, available for 10 cases with intervals ranging from 5 days to 42 months (mean: 15 mo), disclosed one patient dying of operative complications 5 days after biopsy and nine patients with no evidence of disease progression RDD should be considered in the differential diagnosis of inflammatory lesions of the CNS. Our study suggests that this entity may have been misdiagnosed in the past as plasma cell granuloma or inflammatory pseudotumor.
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），是一种影响淋巴结的特发性组织细胞增生性疾病。尽管已报道在不同部位有结外受累情况，但中枢神经系统（CNS）表现，尤其是在无淋巴结疾病的情况下并不常见。我们报告了11例原发性中枢神经系统RDD病例，无其他部位受累的证据。这些病例包括7名男性和4名女性，年龄在22至63岁之间（平均：41岁）。患者表现为头痛、癫痫发作、麻木或截瘫。8例累及颅腔，3例累及椎管。病变最常位于轴外且以硬脑膜为基底。仅1例出现在中枢神经系统实质内。组织学上，病变由数量不等的淡染组织细胞组成，吞噬现象常被背景中广泛的淋巴浆细胞浸润和纤维化所掩盖。针对病原体的特殊染色为阴性。通过免疫组化分析，特征性组织细胞S100蛋白和CD68呈阳性，CD1a呈阴性。治疗包括手术活检或切除。10例患者有随访，随访间隔从5天至42个月不等（平均：15个月），结果显示1例患者活检后5天死于手术并发症，9例患者无疾病进展迹象。在中枢神经系统炎性病变的鉴别诊断中应考虑RDD。我们的研究表明，该疾病过去可能被误诊为浆细胞肉芽肿或炎性假瘤。
