Department of Radiology, Sri Sathya Institute of Higher Medical Sciences, EPIP area, Whitefield, Bangalore, Karnataka, India.
Br J Radiol. 2011 Jul;84(1003):e138-41. doi: 10.1259/bjr/15772106.
We report the first case of extensive involvement of isolated intracranial Rosai-Dorfman's disease (RDD) in a child. Our case is unique because it presents with involvement of the middle cranial fossa, cavernous sinus, pituitary gland, orbit, ethmoid and sphenoid sinuses. Previous cases of intracranial RDD in children have reported separate involvement of cavernous sinus, suprasellar region, and frontal and petroclival regions. Involvement of the pituitary gland has so far not been reported. A 14-year-old male presented with a medical history of loss of vision, raised erythrocyte sedimentation rate (ESR), and abnormal prolactin and cortisol levels. Radiologically the diagnosis was meningioma. The histopathological diagnosis was RDD with emperipolesis and S-100 positivity. RDD is a histiocytic proliferation of unknown aetiology, which commonly affects lymph nodes. Uncommonly it involves the extranodal sites and rarely the central nervous system (CNS). 80 cases of RDD involving CNS have been reported in the literature, and only 5 were in children. Although the definitive diagnosis of RDD disease is based on the histopathology report, it should be included in the differentials of a lesion mimicking meningioma especially in children.
我们报告了首例孤立性颅内 Rosai-Dorfman 病（RDD）广泛受累的病例。我们的病例很独特，因为它涉及中颅窝、海绵窦、垂体、眼眶、筛窦和蝶窦。以前儿童颅内 RDD 的病例报告分别涉及海绵窦、鞍上区、额骨和岩斜区。目前尚未报道垂体受累。一名 14 岁男性，有视力丧失、红细胞沉降率升高（ESR）和异常催乳素和皮质醇水平的病史。放射学诊断为脑膜瘤。组织病理学诊断为伴有 emperipolesis 和 S-100 阳性的 RDD。RDD 是一种病因不明的组织细胞增生症，通常累及淋巴结。它很少累及结外部位，很少累及中枢神经系统（CNS）。文献中已报道了 80 例涉及 CNS 的 RDD 病例，其中只有 5 例发生在儿童。虽然 RDD 疾病的明确诊断基于组织病理学报告，但在儿童中，特别是在类似于脑膜瘤的病变中，应将其纳入鉴别诊断。
