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[Case report of Rosai-Dorfman disease mimicking pachymeningitis]
Yoshimasa Kinoshita1, Hideoki Yasukouchi, Eichi Tsuru
1Department of Neurosurgery, Munakata Suikokai General Hospital, 341-1 Fukuma, Munakata, Fukuoka 811-3298, Japan. yosimasa@suikokai.or.jp
Abstract:
Rosai-Dorfman disease (RDD) is a rare idiopathic histioproliferative disease affecting the lymph nodes. Extranodal sites may be involved and occasionally represent the initial or sole manifestation of the disease. Central nervous system manifestations especially are exceedingly rare. We reported a case of isolated, intracranial, dural-based RDD. A 69-year-old male, admitted with a second generalized convulsion, was examined by high-resolution MR images that revealed a thickened, stratified dura in the left frontal region with associated cortical edema. The patient underwent craniotomy with subtotal resection of the lesion. Microscopically, with the cytoplasmic staining against S-100 protein the lesion was shown to consist of proliferative histiocytes exhibiting emperipolesis. The histopathological diagnosis was compatible with RDD. The diagnosis of RDD mimicking pachymeningitis is presented, and the previous reported cases of intracranial RDD are reviewed.
Insights
Rosai-Dorfman disease (RDD), a rare histioproliferative condition, can manifest in the central nervous system. This case highlights an isolated intracranial dural-based RDD mimicking pachymeningitis.
Area of Science:
- Neurology
- Pathology
- Oncology
Background:
- Rosai-Dorfman disease (RDD) is a rare histioproliferative disorder typically affecting lymph nodes.
- Extranodal involvement is known, but central nervous system (CNS) RDD is exceptionally uncommon.
- This case focuses on an isolated, intracranial, dural-based presentation of RDD.
Observation:
- A 69-year-old male presented with seizures.
- High-resolution MRI revealed a thickened, stratified dura with cortical edema in the left frontal region.
- Surgical resection showed proliferative histiocytes with emperipolesis, positive for S-100 protein.
Findings:
- Histopathological examination confirmed Rosai-Dorfman disease.
- The intracranial lesion mimicked pachymeningitis.
- This represents a rare instance of isolated dural RDD.
Implications:
- Highlights the importance of considering RDD in the differential diagnosis of dural-based intracranial masses.
- Contributes to the understanding of rare CNS manifestations of Rosai-Dorfman disease.
- Reviews previously reported cases of intracranial RDD to aid future diagnoses.
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