Jamali Elena, Sharifi Guive, Ghafouri-Fard Soudeh, Bidari Zerehpoosh Farahnaz, Yazdanpanahi Mahmood, Taheri Mohammad
Department of Pathology, Loghman Hakim Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Skull Base Research Center, Loghman Hakim Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Front Surg. 2022 Mar 21;9:766840. doi: 10.3389/fsurg.2022.766840. eCollection 2022.
Rosai-Dorfman disease (RDD) usually presents with painless cervical lymphadenopathy during the two first decades of life, with or without extranodal involvement. Exclusive extranodal manifestation, and especially central nervous system (CNS) involvement, is uncommon. The etiology remains unknown and definitive diagnosis is based on characteristic histopathological and immunohistochemical features showing S100(+) CD1a(-) RDD type histiocytes with emperipolesis. Most CNS cases are dural-based masses.
Herein, we present a case of RDD in an 8-year-old boy, who presented with multiple huge intraventricular masses.
Regarding rare CNS involvement and lack of established evidence-based therapeutic approaches, reports of any individual case can supply further beneficial data concerning treatment approaches and long-term effectiveness of therapeutic strategies.
罗萨伊-多夫曼病（RDD）通常在生命的头两个十年出现无痛性颈部淋巴结病，有或无结外受累。单纯结外表现，尤其是中枢神经系统（CNS）受累并不常见。病因尚不清楚，确诊基于特征性组织病理学和免疫组化特征，显示具有吞噬现象的S100（+）CD1a（-）RDD型组织细胞。大多数中枢神经系统病例是基于硬脑膜的肿块。
在此，我们报告一例8岁男孩的RDD，其表现为多个巨大的脑室内肿块。
鉴于罕见的中枢神经系统受累以及缺乏既定的循证治疗方法，任何单个病例的报告都可以提供有关治疗方法和治疗策略长期有效性的更多有益数据。
