caa a22 4500 606214186 CHVBK 20210128101051.0 cr unu---uuuuu 210128e20150901xx s 000 0 eng 10.1007/s00701-015-2511-8 doi (NATIONALLICENCE)springer-10.1007/s00701-015-2511-8 Rosai-Dorfman disease involving the central nervous system: seven cases from one institute [Elektronische Daten] [Yongji Tian, Junmei Wang, Mingtao Li, Song Lin, Guihuai Wang, Zhen Wu, Ming Ge, Benoit Pirotte] 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 (6male/1female, 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. Springer-Verlag Wien, 2015 Central nervous system nationallicence Children nationallicence Rosai-Dorfman disease nationallicence Sinus histiocytosis with massive lymphadenopathy nationallicence SLC29A3 gene nationallicence Tian Yongji Department of Neurosurgery, Beijing TianTanHospital, Capital Medical University, Beijing, People's Republic of China aut Wang Junmei Department of Neuropathology, Beijing Neurosurgical Institute, 100050, Beijing, China aut Li Mingtao Department of Neurosurgery, Beijing TianTanHospital, Capital Medical University, Beijing, People's Republic of China aut Lin Song Department of Neurosurgery, Beijing TianTanHospital, Capital Medical University, Beijing, People's Republic of China aut Wang Guihuai Department of Neurosurgery, Beijing TianTanHospital, Capital Medical University, Beijing, People's Republic of China aut Wu Zhen Department of Neurosurgery, Beijing TianTanHospital, Capital Medical University, Beijing, People's Republic of China aut Ge Ming Department of Neurosurgery, Beijing TianTanHospital, Capital Medical University, Beijing, People's Republic of China aut Pirotte Benoit Department of Neurosurgery, CHIREC Hospitals, rue Froissart 38, 1040, Brussels, Belgium aut Acta Neurochirurgica Springer Vienna 157/9(2015-09-01), 1565-1571 0001-6268 157:9<1565 2015 157 701 https://doi.org/10.1007/s00701-015-2511-8 text/html Onlinezugriff via DOI BK010053 XK010053 XK010000 Metadata rights reserved Springer special CC-BY-NC licence nationallicence 1 research-article jats NATIONALLICENCE NATIONALLICENCE NL-springer NATIONALLICENCE 856 40 https://doi.org/10.1007/s00701-015-2511-8 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Tian Yongji Department of Neurosurgery, Beijing TianTanHospital, Capital Medical University, Beijing, People's Republic of China aut NATIONALLICENCE 700 1- Wang Junmei Department of Neuropathology, Beijing Neurosurgical Institute, 100050, Beijing, China aut NATIONALLICENCE 700 1- Li Mingtao Department of Neurosurgery, Beijing TianTanHospital, Capital Medical University, Beijing, People's Republic of China aut NATIONALLICENCE 700 1- Lin Song Department of Neurosurgery, Beijing TianTanHospital, Capital Medical University, Beijing, People's Republic of China aut NATIONALLICENCE 700 1- Wang Guihuai Department of Neurosurgery, Beijing TianTanHospital, Capital Medical University, Beijing, People's Republic of China aut NATIONALLICENCE 700 1- Wu Zhen Department of Neurosurgery, Beijing TianTanHospital, Capital Medical University, Beijing, People's Republic of China aut NATIONALLICENCE 700 1- Ge Ming Department of Neurosurgery, Beijing TianTanHospital, Capital Medical University, Beijing, People's Republic of China aut NATIONALLICENCE 700 1- Pirotte Benoit Department of Neurosurgery, CHIREC Hospitals, rue Froissart 38, 1040, Brussels, Belgium aut NATIONALLICENCE 773 0- Acta Neurochirurgica Springer Vienna 157/9(2015-09-01), 1565-1571 0001-6268 157:9<1565 2015 157 701