Intracranial Rosai–Dorfman disease

Huang, Bo; Zong, Min‐Hua; Zong, Wen; Sun, Yan; Zhang, Hua; Zhang, Hong Bo

doi:10.1016/j.jocn.2015.12.046

Cited by 21 publications

(29 citation statements)

References 24 publications

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“…In our case, the histiocytes positively stain for S-100 and CD68 and negatively for CD1a that were consistent with the diagnosis of RDD. 11 Recent studies have identified recurrent mutation in MAPK/ERK pathway genes in LCH, Erdheim-Chester disease and rarely in some RDD cases. Distinct mutation of MAPK/ERK pathway genes such as BRAF causes ERK over-activation and unrestrained cell proliferation in tumor.…”

Section: Rddmentioning

confidence: 99%

Isolated intracranial Rosai-Dorfman disease in an adult man: Report of a rare case

et al. 2020

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Background: Isolated intracranial Rosai-Dorfman disease (RDD) is an extremely rare, idiopathic histo-proliferative disorder. RDD is associated with the proliferation of histiocytes and emperipolesis. Case Presentation: we report a case with isolated intracranial RDD. A 47-year- old man presented with a dizziness, falling, and then secondary generalized seizure, hemiparesis and right hemisensory deficit. This case preoperatively was misdiagnosed with meningioma. Histopathological examination revealed pale histiocytes displaying emperipolesis which were positive for S-100 and CD68 proteins and negative for CD1a marker. BRAF V600E mutation was negative. Conclusion: In this case, total resection was performed and clinical symptoms were regressed completely.

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Section: Rddmentioning

confidence: 99%

Isolated intracranial Rosai-Dorfman disease in an adult man: Report of a rare case

et al. 2020

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“…Following surgical management, most lesions remain stable or regress. Adjuvant radiotherapy, corticosteroids or chemotherapy have been used for patients with residual tumour or persistent neurological symptoms despite surgery [7]. In our case, the child was treated with 4 weeks of oral prednisolone following the operation.…”

Section: Discussionmentioning

confidence: 98%

A young girl with headache and partial ptosis

Tan

Chan

Rimmer

et al. 2017

Journal of Clinical Neuroscience

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“…RDD is a histioproliferative disorder that mainly involves cervical lymphadenopathy 7 , the disease was first described in 1965 1 , predominantly affecting children and young adults and characterizing massive painless bilateral cervical lymphadenopathy with fever, raised white blood count (WBC) count, high ESR, and polyclonal hypergammaglobulinemia 8 .…”

Section: Discussionmentioning

confidence: 99%

“…Many of these histiocytes contain intact hematopoietic cells, mostly lymphocytes within their cytoplasm, a phenomenon known as emperipolesis 2,3,9 . These histiocytes are positive for S-100 protein and CD-68 and negative for CD1a based on immunohistochemical examination 2,7,10 . Literature describes there may be association of episten bar virus or herpes simplex virus (HSP)-VI associated with this condition 2 .…”

Section: Discussionmentioning

confidence: 99%

Isolated intracranial Rosai Dorfman Disease without nodal involvement.

Aziz¹,

Raja²,

Khimani³

et al. 2019

Int. j. endorsing health sci. res.

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Background: Rosai Dorfman disease (RDD) is a benign lymphohistiocytosis that often involves lymph nodes and present as massive painless lymphadenopathy with sinus histiocytosis. Usually systemic involvement and with rare intracranial and extremely rarely intracranial involvement without the involvement of lymphadenopathy. Case Presentation: We presented a case of 60 years old female with seizures and left side weakness and no lymphadenopathy. The magnetic resonance imaging (MRI) revealed contrasting right frontal homogenously enhancing convexity mass. Management & Results: The patient was kept on antiepileptic medications but soon presented with fits and slight expansion in frontal mass. Surgery was performed (right frontal craniotomy), the mass was surgically resected and biopsy indicated RDD. Conclusion: By now only seven of such cases are reported and prognosis of the disease is not poor if surgically treated however other measures including radiotherapy, chemotherapy, steroids are not very effective for treating the disease. And due to the rarity of disease suspicion of such disease should always be kept as a key differential in homogenously enhancing lesions with dural involvement with or without lymphadenopathy.

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Intracranial Rosai–Dorfman disease

Cited by 21 publications

References 24 publications

Isolated intracranial Rosai-Dorfman disease in an adult man: Report of a rare case

Isolated intracranial Rosai-Dorfman disease in an adult man: Report of a rare case

A young girl with headache and partial ptosis

Isolated intracranial Rosai Dorfman Disease without nodal involvement.

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