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)

Rosai-Dorfman disease is a rare idiopathic histioproliferative disease affecting the lymph nodes. Extranodal involvement has also been recognized, but central nervous system manifestations are extremely rare. Only 12 patients with intracranial involvement have been reported previously, and they all presented with clinical and radiological findings suggestive of meningioma. We report multiple meningeal nodules in a patient presenting with seizures whose pathological findings at surgery confirmed the diagnosis of Rosai-Dorfman disease. A review of all previously reported intracranial lesions is presented.
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PMID:Rosai-Dorfman disease mimicking multiple meningioma: case report. 764 1

This report documents an unusal case of extranodal sinus histiocytosis (Rosai-Dorfman disease) which produced a solitary intracranial mass lesion that was diagnosed clinically and radiologically as a meningioma. Morphological features have been described and literature extensively reviewed.
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PMID:Rosai-Dorfman disease presenting as a meningioma. 925 99

Rosai-Dorfman disease is an idiopathic histiocytic disorder of lymph nodes and extranodal sites. Central nervous system manifestations of this disease are rare, and to our knowledge only 16 cases of intracranial involvement have been reported previously. Intracranial Rosai-Dorfman disease clinically and radiologically resembles meningioma, and histologic examination is essential for a definitive diagnosis. We report two cases of isolated, intracranial, dural-based Rosai-Dorfman disease, review the literature, and discuss the differential diagnoses of this lesion.
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PMID:Isolated intracranial involvement in Rosai-Dorfman disease: a report of two cases and review of the literature. 949 60

Rosai-Dorfman disease, first described in 1969, is a rare idiopathic histioproliferative disease affecting the lymph nodes. Typical clinical features include bilateral painless lymphadenopathy, fever and polyclonal hypergammaglobulinemia. In approximately 43% of cases, extranodal sites may be involved and occasionally represent the initial or sole manifestation of the disease. Central nervous system manifestations are exceedingly rare, and only 17 cases have been recorded, among which there are merely seven isolated intracranial cases without concurrent nodal or other extranodal involvement. Herein, we report a 38-year-old male presenting with generalized tonic-clonic seizure and radiological findings indicative of meningioma. Complete physical examinations and laboratory surveys demonstrate the absence of involvement at other body sites. Microscopically, the lesion consists of proliferative histiocytes exhibiting emperipolesis coupled with the characteristic cytoplasmic staining against S-100 protein. The differential diagnosis of polymorphic inflammatory meningioma-mimicking masses is discussed, and a review of previously reported intracranial Rosai-Dorfman disease is presented.
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PMID:Isolated intracranial Rosai-Dorfman disease: case report and literature review. 970 47

Sinus histiocytosis with massive lymphadenopathy was first described in 1969 in lymphnodes by Rosai and Dorfman ad subsequently in other organs including skin, bone ad soft tissue. Extranodal involvement has also been recognised, but central nervous system manifestations are extremely rare and clinical and radiological findings are suggestive of meningioma. We report a meningeal nodule in a young woman, clinically diagnosed as possible meningioma. A review of literature of previously reported intracranial lesions is presented.
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PMID:[Intracranial location of a case of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) mimicking meningioma]. 979 3

Rosai-Dorfman disease (RDD) is a rare idiopathic histoproliferative disease affecting the systemic lymph nodes. Although an extranodal lesion has also been recognized, central nervous system involvement is extremely rare. To the authors' knowledge, only 20 cases of intracranial lesions have been reported previously. Intracranial RDD is clinically and radiologically difficult to distinguish from meningioma, and histological examination is essential for a definitive diagnosis. The authors treated a large frontal lobe tumor associated with multiple meningeal nodules in a 67-year-old patient presenting with diplopia and headache. Radiological examination indicated that the mass was an inflammatory lesion rather than a meningioma. Microscopically the lesion consisted of mixed inflammatory infiltrate with marked emperipolesis, which is characteristic of RDD. A review of the literature and a discussion of the differential diagnosis of this rare lesion are also presented.
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PMID:Rosai-Dorfman disease presenting multiple intracranial lesions with unique findings on magnetic resonance imaging. Case report. 1043 26

A 45-year-old woman presented with an isolated, contrast-enhancing brain lesion in white matter of the right frontal lobe, preoperatively thought to be either a primary brain neoplasm or metastasis. The lesion was demonstrated by histology and immunohistochemistry to be Rosai-Dorfman disease. Central nervous system (CNS) manifestations of this disease are rare. There have been 27 cases of intracranial involvement reported previously. All of them have been dural-based, where the disease clinically and radiologically resembles meningioma. To our knowledge, this is the first case of an isolated intraparenchymal CNS lesion without dural attachment, where the clinical and radiological features resembled an intraparenchymal glial neoplasm, lymphoma or metastatic tumor.
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PMID:Primary intracerebral rosai-dorfman disease: a case report. 1093 Jan 3

Rosai-Dorfman disease is a well-recognized clinicopathological entity, which in rare cases affects the central nervous system, where it mimics meningioma. We describe three cases and review the literature. Histological and immunohistochemical confirmation is essential for definitive diagnosis. In addition to emperipolesis (lymphophagocytosis), reactivity for S-100 and CD68 and nonreactivity for CD-la immunostaining are characteristic features of this histioproliferative disease. In contrast to meningioma, this tumor usually occurs in young males and infiltrates the brain parenchyma.
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PMID:Meningeal Rosai-Dorfman disease: report of three cases and literature review. 1151 74

Two patients presenting with recurrent visual impairment due to relapsing intracranial Rosai-Dorfman disease are described. In both patients a preoperative diagnosis of meningioma was made. Histological examination disclosed the characteristic picture of S100 and CD68 positive histiocytosis with prominent lymphophagocytosis. In both patients complete tumour removal by surgery was impossible with residual tissue being the origin of relapsing disease. Low dose radiation led to partial recovery of vision and resolution of the intracranial mass. Review of the literature on intracranial Rosai-Dorfman disease leads to the suggestion that postoperative radiotherapy may be advisable in all cases.
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PMID:Relapsing intracranial Rosai-Dorfman disease. 1156 Oct 44

Recently, a number of neoplastic and nonneoplastic entities have been reported that radiographically and clinically mimic meningiomas. Because these lesions occur infrequently and may resemble a meningioma during intraoperative analysis, they may not be considered in the differential diagnosis. This review (and case illustrations) considers some of the newly recognized and notable lesions that can mimic meningiomas, including solitary fibrous tumors, gliosarcomas, leiomyosarcomas, hemangiopericytomas, melanocytomas, Hodgkin's disease, plasmacytomas, inflammatory pseudotumors, neurosarcoidosis, plasma cell granulomas, Rosai-Dorfman disease, Castleman's disease, xanthomas, rheumatoid nodules, and tuberculomas. Awareness that these lesions involve the dura may facilitate intraoperative recognition and, in some cases, preclude unnecessary additional surgery.
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PMID:Dural lesions mimicking meningiomas. 1251 91


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