Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019625 (Rosai-Dorfman disease)
 763 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This article reviews the most frequent extra-axial tumors of the central nervous system, from the most common meningioma to some uncommon conditions, like Rosai-Dorfman disease, focusing on imaging techniques, pearls, and pitfalls as well as a more practical approach.
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PMID:Extraparenchymal Lesions in Adults. 2771 97

Rosai-Dorfman disease (RDD) is a rare condition similar to lymphomas, presenting with cervical lymphadenopathy in young adults. Extra-nodal involvement is relatively common but involvement of the central nervous system (CNS) is rare. Cranial RDD presents with symptoms of raised intracranial pressure, focal or generalised seizures, while spinal RDD presents with pain, peripheral neurological deficits and radiculopathy. In contrast to other similar neoplastic or degenerative conditions affecting the CNS, RDD is a benign, non-infective, granulomatous disorder. Radiologically cranio-spinal RDD often mimics commoner dural-based lesions like meningioma, with only subtle radiological differentiating findings on Magnetic Resonance Imaging (MRI). The histopathology of RDD is diagnostic. Surgical excision is preferred modality of treatment. However, adjuvant therapies like steroids and radiation may help controlling residual or recurrent disease. There are multiple sporadic reports and short case publications in the literature, often focusing on a particular aspect of RDD. In this study, authors aim to present five cases of craniospinal RDD, and comprehensive review of literature and highlight neurological complications of systemic RDD.
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PMID:Cranio-spinal Rosai Dorfman disease: case series and literature review. 2853 72

Inflammatory myofibroblastic tumor (IMT) is a rare tumor in the central nervous system (CNS), mostly being extracranial. Approximately 100 sporadic cases have been reported in the literature. The rarity of the tumor, its various histopathological characteristics, and its variable aggressive course render it difficult to diagnose and treat. IMT is generally a histological diagnosis which is rarely suspected preoperatively. It mimics other intracranial tumors such as giant cell tumor, hemangiopericytoma, anaplastic meningioma, plasmacytoma, and lymphoma. Rarely, it can present with a clinical picture which mimics a benign infective process, Rosai-Dorfman disease, or an idiopathic hypertrophic pachymeningitis. High index of suspicion is required as total resection of this lesion is mandatory to prevent recurrence. Here, we describe a case of a 10-year-old child which initially presented with clinical features mimicking chronic suppurative otitis media and radiological presentation of a small intracranial abscess. He was initially treated by an ENT surgeon who started him on intravenous antibiotics, but the patient was lost to follow up. He returned after 2 months with a large lesion at the same location. Histological examination revealed multiple spindle cells with plasma cells and lymphocytes scattered among these spindle cells. The spindle cells were immunopositive for smooth muscle actin and negative for epithelial membrane antigen, S100, and CD34.
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PMID:Central Nervous System Inflammatory Myofibroblastic Tumor Masquerading as Chronic Suppurative Otitis Media. 2890 84

Sinus histiocytosis with massive lymphadenopathy, or Rosai-Dorfman disease (RDD), was first described in 1969 as a reactive condition of unknown etiology that is characterized by a proliferation of histiocytes exhibiting emperipolesis of both lymphocytes and plasma cells. It usually presents with painless cervical lymphadenopathy either with or without extranodal manifestations. Intracranial involvement of this disease is extremely rare. Intracranial RDD occurs in <5% of all patients with extranodal disease. Here, we report a case of RDD with isolated intracranial involvement. A 67-year-old male presented with a long-standing headache, retro-ocular pain, and progressive visual loss of the left eye. Magnetic resonance imaging showed features of optic nerve meningioma. The histopathology revealed sheets of histiocytes displaying emperipolesis. These histiocytes were S100 positive; however, a CD1a and epithelial membrane antigen were negative.
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PMID:A Rare Case of Isolated Intracranial Rosai-Dorfman Disease Mimicking Optic Nerve Meningioma: A Case Report and Literature Review. 3028 62

Rosai-Dorfman disease (RDD) is a benign histioproliferative disorder characterized by generalized lymphadenopathy, weakness, anemia, and rarely extranodal involvement. The disease affects the central nervous system (CNS) very rarely and at a relatively older age. Isolated intracranial involvement is very rare. RDD tends to present as dural masses mimicking meningioma, though very rare case reports describe isolated parenchymal involvement in the CNS. Only one case of intraventricular RDD has been described so far to the best of our knowledge. We present a very rare presentation of this disease as an isolated intraventricular presentation in the region of the atria of the lateral ventricles.
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PMID:Isolated Intraventricular Rosai-Dorfman Disease. 3045 19

Rosai-Dorfman disease (RDD) with isolated central nervous system (CNS) involvement is an extremely rare disease. Most RDD of the CNS present as dural-based mass mimicking meningioma and other common lesions, which makes preoperative accurate diagnosis of great difficulty. We searched the pathology database in our hospital and 3 cases of RDD with isolated CNS involvement were finally included in our study. Radiological and clinical findings of these three cases were retrospectively analyzed. The lesions of 2 cases were dura-based against the cerebral convexity, presenting as a sheet-shaped thickened dura mater, another case was located just across the cerebral falx, the dural display in the center was intact. The 3 cases showed low signal intensity on T2-weighted image, obviously enhanced, significantly surrounding edema and finger-like protuberance but no invasion of the brain parenchyma or no sign of hyperplasia or sclerosis of the surrounding cranial bones. In conclusion, when we come across a disease that mimicking meningioma, especially when it manifests as the above radiological features, we should considered it might be a kind of proliferative disease of the meninges, such as RDD.
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PMID:Radiological and clinical findings of isolated meningeal Rosai-Dorfman disease of the central nervous system. 3108 64


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