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以硬脑膜及脑实质病变形式出现的孤立性中枢神经系统罗萨伊-多夫曼病。

Isolated Rosai Dorfman disease of the central nervous system presenting as dural-based and intraparenchymal lesions.

作者信息

Sundaram C, Uppin S G, Prasad B C M, Sahu B P, Devi M U, Prasad V S S V, Purohit A K

机构信息

Department of Pathology, Nizam's Institute of Medical Sciences, Hyderabad, India.

出版信息

Clin Neuropathol. 2005 May-Jun;24(3):112-7.

PMID:15943162
Abstract

OBJECTIVE

To report three patients with isolated Rosai Dorfman disease of the central nervous system.

CASE REPORTS

We report two patients with dural-based lesions diagnosed imageologically as meningiomas, and one patient with multiple intraparenchymal lesions diagnosed imageologically as lymphoma. Two patients were males and one was female. All were above 35 years of age with no nodal or other system involvement. The diagnosis was established on surgically excised masses during histopathology. One patient died due to unrelated causes 10 years later and the other two are symptom-free at 21.5- and 11.5-year-follow-up.

CONCLUSIONS

Awareness of the entity and consideration in the differential diagnosis of dural-based/intraparenchymal lesions is necessary for diagnosis and prognosis.

摘要

目的

报告3例中枢神经系统孤立性Rosai-Dorfman病患者。

病例报告

我们报告了2例影像学诊断为脑膜瘤的硬膜病变患者,以及1例影像学诊断为淋巴瘤的多发脑实质内病变患者。2例为男性,1例为女性。所有患者年龄均超过35岁,无淋巴结或其他系统受累。通过组织病理学检查手术切除的肿块确诊。1例患者10年后因无关原因死亡,另外2例在21.5年和11.5年的随访中无症状。

结论

认识该疾病实体并在硬膜病变/脑实质内病变的鉴别诊断中加以考虑,对诊断和预后至关重要。

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