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Rosai-Dorfman disease involving the central nervous system: seven cases from one institute

  • Clinical Article - Brain Tumors
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Abstract

Background

Rosai-Dorfman disease (RDD) is a rare, idiopathic, non-neoplastic histioproliferative disease. Central nervous system (CNS) manifestations are extremely rare. Its low incidence and unknown etiology restrict early diagnosis and optimal therapy.

Methods

In the 1995–2013 period, seven CNS-RDD patients with intracranial and/or spinal lesions were retrospectively analyzed, including the clinical data, laboratory and imaging results, treatment applied and outcome. All seven case samples were screened for the SLC29A3 gene mutation, and the literature was reviewed.

Results

Seven RDD patients (6 male/1 female, aged between 7 and 68) with CNS manifestations are reported. Five of the seven patients (71.4 %) had intracranial lesions (1 with skull erosion and 1 with multiple lesions mimicking meningiomas), and two (28.6 %) had spinal subdural lesions. The patients’ symptoms included headaches, seizures, visual loss, epileptoid convulsions in the lower legs, fever, spastic paraparesis and paraplegia. An elevated erythrocyte sedimentation rate (ESR) was detected in five of the seven cases. The diagnosis was confirmed by immunohistochemical staining revealing that the characteristic histiocytes were positive for the S100 protein and CD68 and negative for CD1a. All patients were operated on: three recovered completely, two were partially rehabilitated, and two died. No SLC29A3 gene mutations were found in any of the seven samples.

Conclusion

This short series suggests the following: (1) RDD should be included in the differential diagnosis of lesions mimicking intracranial/spinal meningiomas or inflammatory lesions, especially in children; (2) the definitive diagnosis is based on histopathology and immunocytochemistry; (3) surgical resection seems to be the most effective therapy; (4) the exact etiology and adjuvant therapy for relapsing/incompletely resected lesions remain to be established.

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Acknowledgments

Dr. Yongji Tian (first author) was partially supported by the National Natural Science Foundation of China (No. 30900479) , Beijing Nova-Plan Program (No. 2010B121), National Key Technology Research and Development Program of the Ministry of Science and Technology of China (2013BAI09B03) and BIBD-PXM 2013_014226_07_000084.

Conflicts of interest

None.

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Authors and Affiliations

  1. Department of Neurosurgery, Beijing TianTanHospital, Capital Medical University, Beijing, People’s Republic of China

    Yongji Tian, Mingtao Li, Song Lin, Guihuai Wang, Zhen Wu & Ming Ge

  2. China National Clinical Research Center for Neurological Diseases (NCRC-ND); Center for Brain Tumor, Beijing Institute for Brain Disorders; Beijing Key Laboratory for Brain Tumor, Beijing, 100050, People’s Republic of China

    Yongji Tian, Mingtao Li, Song Lin, Guihuai Wang, Zhen Wu & Ming Ge

  3. Department of Neuropathology, Beijing Neurosurgical Institute, Beijing, 100050, China

    Junmei Wang

  4. Department of Neurosurgery, CHIREC Hospitals, rue Froissart 38, 1040, Brussels, Belgium

    Benoit J. M. Pirotte

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  1. Yongji Tian
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Correspondence to Ming Ge.

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Tian, Y., Wang, J., Li, M. et al. Rosai-Dorfman disease involving the central nervous system: seven cases from one institute. Acta Neurochir 157, 1565–1571 (2015). https://doi.org/10.1007/s00701-015-2511-8

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  • DOI: https://doi.org/10.1007/s00701-015-2511-8

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