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

Sinus histocytosis with massive lymphadenopathy, also known as Rosai-Dorfman Disease (RDD), is an idiopathic histiocytic proliferation affecting lymph nodes. Although extranodal involvement has been reported in diverse sites, central nervous system (CNS) manifestation, particularly in the absence of nodal disease is uncommon. We report 11 cases of RDD primary to the CNS without evidence of other sites of involvement. The cases included 7 males and 4 females ranging in age from 22 to 63 years (mean: 41 y). The patients presented with headaches, seizures, numbness, or paraplegia. Eight cases involved the cranial cavity and three cases, the spinal canal. Lesions were most often extra-axial and dura based. Only one presented in the CNS parenchyma. Histologically, the lesions consisted of variable numbers of pale-staining histocytes with emperipolesis often overshadowed by extensive lymphoplasmacytic infiltrates and fibrosis in the background. Special stains for organisms were negative. By immunohistochemical analysis, the characteristic histiocytes were positive for S100 protein and CD68 and negative for CD1a. Treatment consisted of surgical biopsy or excision. Follow-up, available for 10 cases with intervals ranging from 5 days to 42 months (mean: 15 mo), disclosed one patient dying of operative complications 5 days after biopsy and nine patients with no evidence of disease progression RDD should be considered in the differential diagnosis of inflammatory lesions of the CNS. Our study suggests that this entity may have been misdiagnosed in the past as plasma cell granuloma or inflammatory pseudotumor.
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PMID:Rosai-Dorfman disease isolated to the central nervous system: a report of 11 cases. 1126 22

Rosai-Dorfman disease is an idiopathic histiocytic proliferative disorder characterized by sinus histiocytosis and massive lymphadenopathy. Extranodal involvement has also been reported. We report the case of a 57-year-old woman presenting with seizures, focal motor deficits, and leptomeningeal enhancement on magnetic resonance imaging. Meningeal biopsy revealed Rosai-Dorfman disease. Sinus histiocytosis may mimic meningeal disease, and Rosai-Dorfman disease should be considered in the differential diagnosis of dural-based masses with diffuse leptomeningeal enhancement.
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PMID:Extranodal Rosai-Dorfman disease involving the meninges. 1235 24

The May 2002 COM. A 38-year-old man presented with new onset seizures and a 69-year-old woman presented with bilateral headaches and episodes of syncope. Both were found to have extra-axial masses that were contrast-enhancing and thought to be meningiomas. Both had complete resection. Microscopic examination revealed an inflammatory lesion composed of plasma cells, scattered lymphocytes and numerous large histocytic cells, which exhibited emperi polesis and were CD1 a negative, but positive for CD68 and S100. The diagnosis of Destombes-Rosai-Dorfman Disease (DRDD) was rendered. Both cases had good long-term outcome. The differential diagnosis of inflammatory masses in the dura (plasmacytoma, lymphomas, plasma cell fibroma, angiofollicular hyperplasia [Castleman's-disease] and Langerhan's cell histiocytosis) are discussed.
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PMID:May 2002: 38-year-old man and 69-year-old woman with dural based masses. 1240 40

Rosai-Dorfman disease (RDD) is an idiopathic proliferation of histiocytes that affects the lymph nodes. Central nervous system involvement in the absence of nodal disease is extremely rare. On neuroimaging studies, intracranial RDD appears as solitary or multiple well-circumscribed, dura-based lesions. The authors report on two cases of RDD with locally aggressive features including dural sinus invasion, which to their knowledge has never before been described. A 60-year-old woman presented with progressive dizziness and vertigo that had lasted for 1 week. Cranial computerized tomography and magnetic resonance imaging revealed an extraaxial homogeneous lobulated enhancing mass involving the right occipital lobe and the right cerebellar hemisphere. Invasion of the right transverse sinus was identified on a cerebral digital subtraction angiogram. A 59-year-old man with no prior medical illness experienced progressive weakness of both upper extremities and a partial complex seizure. Magnetic resonance imaging of his brain revealed a well-circumscribed enhancing mass in the left frontal lobe with extension to the right frontal lobe and invasion of the superior sagittal sinus. Both patients underwent resection of their brain masses. Pathological studies identified the disease as RDD in both patients.
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PMID:Rosai-Dorfman disease with dural sinus invasion. Report of two cases. 1579 95

Rosai-Dorfman disease is rare. When it does occur, it usually affects children, and it has a propensity for whites and blacks as opposed to members of other races. We report the case of a 45-year-old Asian Indian woman who presented with painful masses in the area of the axillary, cervical, and inguinal lymph nodes. She had a decade-long history of tonic-clonic seizures and a recent history of digestive complaints and progressive nasal stuffiness. Endoscopic examination of the left nasal cavity revealed the presence of submucosal bulges along the septum and the lateral wall; hypertrophy of the adenoids was also noted. Histologic analysis of lymph node specimens revealed dilated parenchymal sinuses, germinal activity with infiltration of numerous histiocytes (emperipolesis), and chronic inflammatory cells. The patient was diagnosed with Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) with atypical extranodal involvement (the nasal area). An aggressive course of polychemotherapy thrice daily was initiated, but it had no lasting effect, and the patient died 8 months later of an undetermined cause. Our patient's age at the onset of her disease, her race, and the extranodal involvement make this case of Rosai-Dorfman disease unusual and perhaps unique.
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PMID:Rosai-Dorfman disease in an Asian Indian woman with classic generalized lymphadenopathy and nasal obstruction: a case report. 1929 21

We report a rare occurrence of intracranial Rosai-Dorfman disease (RDD), in a young patient with multiple bilateral intracerebral lesions, and discuss her management. RDD is an histiocytic proliferative disorder, which seldom presents with intracranial pathology. Intraparenchymal RDD without concurrent systemic features is rare. This 31-year old woman presented with two focal seizures, but no peripheral lymphadenopathy, and no significant systemic abnormalities. Preoperative imaging showed intrinsic space-occupying lesions in the left frontal lobe, and right parietal and right inferior frontal white matter. She underwent excision of the peripherally located left frontal lesion, and aside from a five-day course of postoperative dexamethasone, received no further active treatment. Histopathological analysis revealed the characteristic features of RDD. Postoperative imaging, taken at 10 weeks and 12 months, demonstrated resolution of all three lesions. This patient had a rare multifocal, bilateral, intracerebral RDD, illustrating that although a histological diagnosis is necessary, further treatment may not be required.
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PMID:Intracerebral multifocal Rosai-Dorfman disease. 2272 7

Extranodal sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) is a non-neoplastic condition that has rarely been reported to involve the central nervous system. This report documents a 28-year-old man with Rosai-Dorfman disease who presented with a seizure and a dural-based mass that was thought to represent a meningioma. Resection showed a lesion marked by large, S-100 protein immunoreactive histiocytic cells with intermixed benign lymphocytes and plasma cells. Emperipolesis with intracytoplasmic lymphocytes and plasma cells was present. Differential diagnostic considerations will be discussed.
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PMID:Dural-based Rosai-Dorfman disease: differential diagnostic considerations. 2528 Oct 34

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

The association between Rosai-Dorfman Disease (RDD) and cancer was reported for the first time in 1984. However, there are still a small number of reports of this association. We describe a 60-year-old woman who presented with a focal onset motor seizure followed by tonic-clonic generalization and persistent headache. Magnetic Resonance Imaging disclosed an irregular hyperintense lesion in T2 and vasogenic edema in the left parietal region. Immunohistochemical analysis of a biopsy fragment was positive for protein S-100 and CD68 and negative for CD1a, compatible with the diagnosis of RDD. She previously had breast cancer six years earlier and had used tamoxifen for two years and anostrozol for three years after diagnosis of cancer. RDD has been already associated with different cancers, such as Hodgkin's lymphoma, non-Hodgkin's lymphoma, follicular lymphoma, melanoma, adenocarcinoma and small cell lung cancer. As far as we know, this is the first report of an association between breast cancer and RDD.
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PMID:Isolated central nervous system Rosai-Dorfman disease and breast cancer: an unusual presentation. 3029 95


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