Chong César, Wong-Achi Xavier, Apolo Cinthya
Departments of Neurosurgery and.
Universidad Espíritu Santo, Samborondón, Ecuador.
J Neurosurg Case Lessons. 2021 Jun 14;1(24):CASE21238. doi: 10.3171/CASE21238.
Rosai-Dorfman-Destombes disease is a rare and heterogeneous entity that has been associated with autoimmune, hereditary, and malignant diseases. There is controversy about its etiopathogenesis, clinical course, and therapeutic management.
The authors report a case of a 61-year-old man with a history of progressive headache without any other symptoms. Magnetic resonance imaging of the brain revealed multiple irregular lesions with an initial diagnostic impression of meningiomatosis. An excisional biopsy was performed, and the pathology report stated the finding was Rosai-Dorfman-Destombes disease.
The uniqueness of this case is its rarity. The isolated intracranial location presents many diagnostic and therapeutic challenges, with radiological and clinical characteristics similar to those of other central nervous system tumors. There is currently no clear evidence of the pathogenesis and therapeutic management of this condition. Follow-up of these patients will help elucidate the natural history of this condition and the benefits of various treatment modalities.
罗萨伊-多夫曼-德斯顿贝斯病是一种罕见且异质性的疾病，与自身免疫性、遗传性和恶性疾病有关。关于其病因、临床病程和治疗管理存在争议。
作者报告了一例61岁男性病例，有进行性头痛病史，无任何其他症状。脑部磁共振成像显示多个不规则病变，初步诊断印象为脑膜瘤病。进行了切除活检，病理报告显示结果为罗萨伊-多夫曼-德斯顿贝斯病。
该病例的独特之处在于其罕见性。孤立的颅内病变带来了许多诊断和治疗挑战，其放射学和临床特征与其他中枢神经系统肿瘤相似。目前尚无关于这种疾病发病机制和治疗管理的确切证据。对这些患者的随访将有助于阐明这种疾病的自然病程以及各种治疗方式的益处。
