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CASE REPORT article

Front. Oncol.
Sec. Neuro-Oncology and Neurosurgical Oncology
Volume 14 - 2024 | doi: 10.3389/fonc.2024.1487835
This article is part of the Research Topic Recent Advances in Diagnosis and Treatment of Brain Tumors: From Pediatrics to Adults View all 23 articles

Pediatric Intraventricular Rosai-Dorfman Disease: Clinical Insights and Surgical Strategies in a Decade-Long Observational Study and Literature Review

Provisionally accepted
Dayuan Liu Dayuan Liu 1*Ning Li Ning Li 1*Yubo Zhu Yubo Zhu 1*Yunxiang Zhong Yunxiang Zhong 1*Guolong Deng Guolong Deng 1*Mingfa Wang Mingfa Wang 1*Caicai Zhang Caicai Zhang 2*Jigao Feng Jigao Feng 1*
  • 1 Second Affiliated Hospital of Hainan Medical University, Haikou, China
  • 2 Hainan Medical University, Haikou, Hainan Province, China

The final, formatted version of the article will be published soon.

    Background: Rosai-Dorfman disease (RDD), or sinus histiocytosis with massive lymphadenopathy (SHML), is a rare benign disorder characterized by the proliferation of histiocytes of uncertain origin. Central nervous system (CNS) involvement, particularly intraventricular, is exceptionally rare and poses significant diagnostic challenges due to its non-specific clinical and radiographic presentation. This study aims to present a case of intraventricular RDD and review existing literature on its clinical features, treatment strategies, and prognosis.We report the case of a five-year-old male with recurrent headaches and epilepsy caused by an intraventricular mass. The mass was surgically resected and histopathological examination was performed to confirm the diagnosis. A comprehensive literature review was conducted to identify similar cases of intraventricular RDD, focusing on clinical features, diagnostic methods, treatment strategies, and outcomes.Results: Histopathological examination of the resected tumor revealed typical features of RDD, including large histiocytes, lymphocyte infiltration, and immunohistochemical positivity for CD68, S-100, and Vimentin. The patient remained asymptomatic ten years post-surgery with no recurrence of epilepsy or tumor. The literature review identified six similar cases, all of which showed favorable outcomes post-surgery, highlighting the self-limiting nature and favorable prognosis of intraventricular RDD following surgical resection.Intraventricular RDD, though rare, should be considered in the differential diagnosis of intraventricular masses in pediatric patients. Surgical resection remains the primary treatment modality, and histopathological confirmation is essential for accurate diagnosis. The prognosis is generally favorable with appropriate surgical intervention, although recurrence can occur, necessitating long-term follow-up. Further research is required to refine diagnostic criteria and explore adjuvant therapies for improved management of this rare CNS disorder.

    Keywords: Rosai-Dorfman disease (RDD), intraventricular tumor, pediatric epilepsy, Central nervous system histiocytosis, Surgery

    Received: 28 Aug 2024; Accepted: 13 Nov 2024.

    Copyright: © 2024 Liu, Li, Zhu, Zhong, Deng, Wang, Zhang and Feng. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

    * Correspondence:
    Dayuan Liu, Second Affiliated Hospital of Hainan Medical University, Haikou, China
    Ning Li, Second Affiliated Hospital of Hainan Medical University, Haikou, China
    Yubo Zhu, Second Affiliated Hospital of Hainan Medical University, Haikou, China
    Yunxiang Zhong, Second Affiliated Hospital of Hainan Medical University, Haikou, China
    Guolong Deng, Second Affiliated Hospital of Hainan Medical University, Haikou, China
    Mingfa Wang, Second Affiliated Hospital of Hainan Medical University, Haikou, China
    Caicai Zhang, Hainan Medical University, Haikou, 571199, Hainan Province, China
    Jigao Feng, Second Affiliated Hospital of Hainan Medical University, Haikou, China

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