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 (RDD) is an idiopathic histiocytic proliferation affecting lymph nodes. Although extranodal involvement has been reported in diverse sites, manifestation in the central nervous system (CNS) is extremely rare, particularly in the brain parenchyma. A 39-year-old male presented with an isolated well-circumscribed brain mass in the right temporal lobe, preoperatively thought to be a meningioma. Histology and immunohistochemistry confirmed that the lesion was RDD. The intraparenchymal brain location of RDD appears to have a benign course. Although the adjuvant therapy is a treatment of choice, surgical resection seems to be the appropriate treatment modality. From the clinical point of view RDD might be an important intracerebral entity because it may mimic other lesions, particularly other histiocytic disorders.
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PMID:Extranodal sinus histiocytosis (Rosai-Dorfman disease) of the brain parenchyma. 1260 63

Rosai-Dorfman disease (RDD), a systemic histioproliferative disorder, was first described by Rosai and Dorfman in 1969. However, only 41 cases involving the central nervous system have been reported. The authors present a rare case of RDD with multiple intracranial and spinal lesions mimicking multiple meningioma. A 59-year-old woman was admitted to our hospital with a history of bilateral visual impairment. MRI demonstrated multiple central nervous system lesions in the suprasellar region, right temporal convexity, left frontal convexity, left cerebello-pontine angle and C5 level of the spinal cord. Preoperative neuro-imaging studies suggested multiple meningioma. Subtotal removal of the suprasellar lesion was performed, followed by gamma knife radiosurgery to the residual lesion and the other intracranial lesions. All intracranial lesions disappeared following gamma knife radiotherapy. The spinal lesion, however, was completely excised one year after the initial operation following the appearance of sensory disturbance. Both intracranial and spinal lesions were diagnosed as RDD on histological examination. No recurrences have been detected on MRI two years after the initial operation. Seeing the good results already achieved by surgical removal of RDD, this case suggests that the combination of surgery and gamma knife radiotherapy might improve further.
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PMID:[Rosai-Dorfman disease presenting with multiple intracranial and intraspinal masses: a case report]. 1465 92

The May 2003 COM. A 57-year-old woman presented with slurring of her speech and right arm weakness. Her past medical history included idiopathic hypertrophic subendocardial stenosis (IHSS), arthritis, asthma, congestive heart failure, hypertension and NIDDM. Neurological examination showed persistent word finding difficulty but her motor and sensory function had essentially returned to normal. Extensive laboratory studies were unrevealing. Imaging studies showed a meningeal lesion over the left posterior parietal lobe and the findings suggested an infectious or inflammatory process. A biopsy of the involved dura and meninges was performed and revealed leptomeningeal Rosai-Dorfman disease. Emperipolesis was noted. The finding of emperipolesis is characteristic of Rosai-Dorfman disease of the leptomeninges, but in 30% of cases, this feature will not be identified. Large pale histiocytes of Rosai-Dorfman disease are immunoreactive for S-100 protein and KP1, but negative for CD1a. The differential diagnosis of a chronic inflammatory infiltrate containing numerous, large histiocytes includes granulomatous diseases such as Wegener graulomatosis and sarcoid, Hodgkin disease, and Langerhans histiocytosis. CNS Rosai-Dorfman most commonly involves patients between 20- and 40-years-old, with a slight male predominance. Approximately 75% of cases are intracranial, whereas 20% involve the spine. Over 90% of CNS Rosai-Dorfman cases involve the leptomeninges and are seen by neuroimaging as a dural-based, contrast-enhancing masses that often elicit vasogenic edema in the underlying brain. Thus, clinically and radiologically, the disease is thought to represent meningioma. Leptomeningeal Rosai-Dorfman disease is considered a benign condition and in most cases surgical resection is the treatment of choice. Although the number of cases in the literature is small, disease progression following surgical resection is uncommon. Little is known regarding the pathogenesis of Rosai-Dorfman disease. Most have suggested that it represents either an autoimmune disease or a reaction to an infectious agent that has yet to be discovered. Currently it is best considered a benign, idiopathic histiocytosis.
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PMID:May 2003: 57-year-old-woman with acute loss of strength in her right upper extremity and slurred speech. 1465 68

A 9-year-old boy presented with a 12-month history of headaches and recent grand mal seizures. Computed tomography and magnetic resonance imaging of the brain revealed an enhancing right frontal convexity lesion. The preoperative diagnosis was meningioma. However, histological examination was diagnostic of Rosai-Dorfman disease. Rosai-Dorfman disease confined to the intracranial compartment is very rare, of 34 reported cases only one presented in the first decade. Optimal treatment has not been established, but complete surgical resection alone seems effective and allows histological distinction from meningioma.
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PMID:Isolated intracranial Rosai-Dorfman disease mimicking meningioma in a child. 1532 36

Sinus histiocytosis with massive lymphadenopathy (SHML), also known as Rosai-Dorfman disease (RDD), is an idiopathic histiocytic proliferation affecting lymph nodes. It is typically characterized by painless cervical lymphadenopathy, fever and weight loss. Although extranodal involvement has been reported in diverse sites, intracranial presentation, particularly in the absence of nodal disease is uncommon. To the best of our knowledge, 48 patients with intracranial masses have been reported previously. A 31-year-old man was admitted to our clinic with a 4-month history of progressive headache. His medical history was unremarkable except for occasional fever. There were not any neurological deficit and weight loss. No lymphadenopathy (particularly bilateral cervical) and extranodal involvement in diverse sites were revealed by physical and radiological examinations. Routine hematological and biochemical studies were normal except for mild leukocytosis and elevated erythrocyte sedimentation rate. The patient underwent magnetic resonance imaging (MRI) testing that revealed an enhancing mass in the left temporal lobe. Preoperative diagnosis was meningioma. The patient underwent a left frontotemporal craniotomy with complete resection of the mass. Histopathology was compatible with RDD. Extranodal RDD is rarely found intracranially. Prognosis is benign especially in the absence of nodal disease. It is clinically and radiologically difficult to distinguish from meningioma, and histological examination is essential for a definitive diagnosis.
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PMID:Isolated intracranial Rosai-Dorfman disease mimicking meningioma. 1558 Oct 22

A 40-year-old male presented with a single generalized tonic-clonic seizure. MRI revealed an enhancing, dural-based, left lateral sphenoid wing lesion suggestive of a meningioma. At microsurgical excision, the lesion was firm and relatively avascular. The histopathological report revealed S-100 positive histiocytic proliferation with lymphophagocytosis (emperipolesis) characteristic of the Rosai-Dorfman disease. The case and its management are discussed.
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PMID:Rosai-Dorfman disease mimicking a sphenoid wing meningioma. 1580 69

Rosai-Dorfman disease (RDD) is an idiopathic, non-neoplastic, lymphoproliferative disorder characterized by sinus histiocytosis and massive lymphadenopathy. When RDD involves the central nervous system the lesion simulates a meningioma. Histological and immunohistochemical confirmation is essential for a definitive diagnosis. In this paper, ten cases of RDD confined to the central nervous system are reported. Another case with orbital RDD was excluded. Nine cases involved the cranial cavity alone; in one, the cervical extradural region was also involved. Treatment consisted of surgical excision or biopsy. Histology and immunohistochemistry revealed a mixed cell population of predominantly mature histiocytes with evidence of emperipolesis and strong positivity for S100 protein in all cases. No recurrence was observed during follow up ranging from three months to eight years.
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PMID:Rosai-Dorfman disease of the central nervous system. 1609 62

Sinus histiocytosis with massive lymphadenopathy (SHML, Rosai-Dorfman disease), is a well-defined histioproliferative disorder. The extranodal type of the disease is not uncommon, but CNS involvement is extraordinary. Moreover, the isolated extranodal intracranial manifestation of the disease is extremely rare. Only a few cases of the latter form have been reported. We present a case of meningioma-like intracranial lesion, which after total resection, pathologically and immunohistochemically proved to be an isolated form of the Rosai-Dorfman disease. No concurrent nodal involvement was found, even after a detailed postoperative diagnostic work-up.Two years follow-up disclosed full recovery from the CNS symptoms, while no other SHML signs were noted. The extranodal form of the Rosai-Dorfman Disease, nowadays has been enlisted in the differential diagnosis of the space occupying intracranial and skull base lesions. However, a number of questions, dealing with the preoperative diagnosis, the surgical strategies, as well as the natural history, the prognosis of the disease, and the possible need for adjunctive therapy, remain obscured. Additional reports are useful to better understand this rare type of disease and may lead to the establishment of a more profitable management.
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PMID:A new clinical entity mimicking meningioma diagnosed pathologically as rosai-dorfman disease. 1717 Oct 57

The authors describe the case of a 44-year-old man with multilevel lumbar spinal Rosai-Dorfman disease (RDD), a rare clinical entity. To the authors' knowledge, there have been only 2 cases of lumbar spinal involvement of RDD (epidural) reported in the literature, and the current case is the third but the only one showing lumbar spinal intradural involvement of RDD. This case of RDD mimicked a meningioma both clinically and radiologically. The patient underwent a procedure in which the tumor was excised, and postoperatively the patient made a clinically acceptable recovery. Vertebral canal involvement of RDD should be considered in the differential diagnosis of vertebral canal tumors. Resection is an acceptable treatment option.
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PMID:Extranodal Rosai-Dorfman disease with multilevel lumbar spinal lesions. 1859 Apr 11

Rosai-Dorfman disease (RDD) is an idiopathic histiocytic proliferation affecting the lymph nodes. Isolated intracranial RDD is rare and usually appears as a well-defined, dural-based lesion without lymphadenopathy. The clinical and radiological features of intracranial RDD are similar to meningioma. Histopathology and immunohistochemistry are essential for a definitive diagnosis. This is a report of a 43-year old male with isolated intracranial RDD, which manifested as a suprasellar meningioma. The clinical, radiological and pathological aspects of the disease are discussed within the context of a review of previously reported cases.
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PMID:Isolated intracranial Rosai-Dorfman disease mimicking suprasellar meningioma: case report with review of the literature. 1883 12


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