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首页> 外文期刊>Child's nervous system: ChNS : official journal of the International Society for Pediatric Neurosurgery >Intracranial Rosai-Dorfman disease in a child mimicking bilateral giant petroclival meningiomas: a case report and review of literature.
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Intracranial Rosai-Dorfman disease in a child mimicking bilateral giant petroclival meningiomas: a case report and review of literature.

机译：模仿双侧巨大石斜坡脑膜瘤的儿童颅内罗萨伊-多夫曼病：一例病例报道并文献复习。

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OBJECTIVES AND IMPORTANCE: Rosai-Dorfman disease (RDD) is a rare but distinctive entity of unknown etiology; isolated intracranial RDD is uncommon. Of 37 reported intracranial RDD cases, only three were reported in children. CLINICAL PRESENTATION: We report an unusual case of a 15-year-old boy presenting with 4 months history of raised intracranial pressure with visual deterioration. Computed tomography and magnetic resonance imaging revealed bilateral petroclival enhancing lesions with cavernous sinus extension mimicking meningioma. However, histological examination was diagnostic of RDD. INTERVENTION: The patient underwent extended right-sided middle fossa approach and near-total tumor removal from petroclival region and cavernous sinus on both sides in two stages 6 weeks apart. CONCLUSION: Ours is the first case of pediatric isolated intracranial RDD presenting with giant bilateral petroclival masses successfully managed with bilateral extended middle fossa approach in two stages. An optimal treatment for RDD is not established, but complete surgical resection alone seems effective.
机译：目的和重要性：罗莎-多夫曼病（RDD）是一种病因不明的罕见但独特的个体。孤立的颅内RDD并不常见。在报告的37例颅内RDD病例中，仅3例是儿童。临床表现：我们报告了一个不寻常的病例，该病例为一个15岁男孩，表现出颅内压升高4个月且视力下降的历史。计算机体层摄影术和磁共振成像显示双侧石斜坡增强病变，并伴有类似于脑膜瘤的海绵窦延伸。但是，组织学检查可诊断为RDD。干预：患者接受了延长的右侧中窝入路，并且在距石坡区和两侧海绵窦近乎完全切除的肿瘤中分两个阶段分6周进行。结论：我们的病例是首例小儿孤立性颅内RDD，其表现为两个阶段均采用双侧扩展中窝窝入路成功治疗的巨大双侧石棺肿块。尚未确定RDD的最佳治疗方法，但仅单纯手术切除似乎有效。

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