Clinicopathological characteristics of multiple intracranial Rosai-Dorfman disease with increased IgG4-positive plasma cells: a report of two cases

Chunhui Zhou; Hongxiang Ren; Liu Hong; Jianning Zhang; Hongxiang Wang

doi:10.5114/fn.2021.112565

eISSN: 1509-572x
ISSN: 1641-4640

Folia Neuropathologica

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4/2021
vol. 59

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abstract:

Case report

Clinicopathological characteristics of multiple intracranial Rosai-Dorfman disease with increased IgG4-positive plasma cells: a report of two cases

Chunhui Zhou

¹

,

Hongxiang Ren

²

,

Liu Hong

³

,

Jianning Zhang

¹

,

Hongxiang Wang

⁴

Department of Neurosurgery, The Sixth Medical Center of PLA General Hospital, Beijing, China
Department of Neurosurgery, China-Japan Friendship Hospital, Capital University of Medical, Beijing, China
Department of Pathology, The Sixth Medical Center of PLA General Hospital, Beijing, China
Department of Neurosurgery, Changhai Hospital, Naval Military Medical University, Shanghai, China

Folia Neuropathol 2021; 59 (4): 409-418

DOI: https://doi.org/10.5114/fn.2021.112565

Online publish date: 2021/12/30

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Rosai-Dorfman disease (RDD) is an uncommon condition characterized by the proliferation of histiocytes and multiple intracranial involvements and it is extremely rare. Here, we present two cases of multiple intracranial RDD mimicking meningioma. These patients underwent surgery for tumour resection and pathological findings revealed an increased number of IgG4-positive plasma cells in RDD. The radiographic appearance and histology may contribute to a diagnostic dilemma, and immunohistochemical and serological examinations are a necessary complement for definitive diagnosis. Treatment protocols pertaining to such types of RDD cases are reviewed. Currently, surgical resection is the most effective therapy, and steroid therapy, radiotherapy, or chemotherapy may be provided as adjuvant treatments in some selected patients.

keywords:

Clinicopathological characteristics of multiple intracranial Rosai-Dorfman disease with increased IgG4-positive plasma cells: a report of two cases

Chunhui Zhou 1 , Hongxiang Ren 2 , Liu Hong 3 , Jianning Zhang 1 , Hongxiang Wang 4

Rosai-Dorfman disease, multiple intracranial lesions, IgG4, diagnosis, treatment

Chunhui Zhou

¹

,

Hongxiang Ren

²

,

Liu Hong

³

,

Jianning Zhang

¹

,

Hongxiang Wang

⁴