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 benign idiopathic histioproliferative disorder usually manifesting as massive painless adenopathy. Extranodal involvement of the Central Nervous System (CNS) mimicking a skull base meningioma is rare. A 42-year-old male presented with painless, progressive left visual loss of 4 months duration. Clinically, he had a left ptosis, proptosis and ophthalmoplegia. Magnetic Resonance Imaging (MRI) of the brain with gadolinium revealed a destructive lesion of the left orbital apex, middle cranial fossa and cavernous sinus. He was treated with corticosteroids and underwent debulking. Pathology showed inflammatory infiltrate in the absence of an infectious agent, emperipolesis and a positive S100 stain was consistent with Rosai-Dorfman disease. As there was no improvement following steroids and debulking, he underwent radiation therapy with significant improvement of his symptoms. Although a rare entity, Rosai-Dorfman disease should be considered in the differential of a skull base lesion.
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PMID:Rosai-Dorfman disease manifesting as intracranial and intraorbital lesion. 1902 Aug 7

Rosai-Dorfman disease is a common benign lymphomatous disorder that involves the lymph nodes and other solid organs. Neurological involvement is rare, and an isolated intracranial Rosai-Dorfman lesion even rarer. Whether isolated intracranial Rosai-Dorfman lesions should be defined as Rosai-Dorfman disease or intradural histiocytosis remains debatable. Although these intracranial masses may radiologically mimic a meningioma, some have diagnostically specific radiological appearances. Because isolated intracranial Rosai-Dorfman lesions are so rarely described their best treatment is controversial. Most patients described in published reports underwent surgery followed in selected cases by adjuvant radiotherapy. Medical therapies achieve conflicting results. Our two case reports, a patient with isolated dural intracranial Rosai-Dorfman lesions and another with multiple intracranial lesions, both without lymphadenopathy, should be of interest to neuroradiologists and neurosurgeons managing similar cases.
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PMID:Intracranial dural histiocytosis. 1963 21

We reviewed the diagnosis and treatment of six patients with CNS Rosai-Dorfman disease (RDD). Lesions were located in the cerebral convexity, middle cranial base, parasaggital, petrous orbit, and thoracic spine. Preoperatively, all the lesions were misdiagnosed as meningioma. Histopathology of all CNS lesions showed a characteristic feature called emperipolesis, where small lymphocytes or plasma cells were engulfed in histiocyte cytoplasm. Total resection of lesions was performed in all cases, and at an average follow-up of 15 months, all patients are alive and well with no evidence of recurrence. Preoperative diagnosis of CNS RDD is challenging. Surgical removal of lesions is an effective treatment. More research is needed to clarify the effectiveness of other treatment options such as radiosurgery and corticosteroid therapy.
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PMID:Rosai-Dorfman disease isolated to the central nervous system: a report of six cases. 1970 67

Sinus histiocytosis with massive lymphadenopathy (SHML), also known as Rosai-Dorfman disease (RDD), is an idiopathic histiocytic disorder of lymph nodes and extranodal sites. Central nervous system (CNS) manifestations, particularly in the absence of nodal disease, are rare. Intracranial RDD clinically and radiologically resembles meningioma, and histologic examination is essential for a definitive diagnosis. We report four patients with RDD primary to the CNS without evidence of other sites of involvement, review the literature, and discuss the clinical manifestations, pathology, treatment and outcome.
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PMID:Primary intracerebral Rosai-Dorfman disease. 2062 63

Rosai-Dorfman disease (RDD), also known as sinus histocytosis with massive lymphadenopathy, is a rare idiopathic histioproliferative disease affecting the lymph nodes. Although extranodal involvement has been reported in diverse sites, central nervous system manifestations, particularly in the absence of nodal disease with clinical and radiological findings suggestive of meningioma, are extremely rare. Histopathology and immunohistochemistry are essential for a positive diagnosis. We report a case of RDD in a patient presenting multiple meningeal nodules with a review of the literature and discussion of differential diagnosis.
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PMID:[Rosai-Dorfman disease mimicking meningiomatosis: a case report]. 2107 26

Rosai-Dorfman disease is a rare, benign, idiopathic histio-proliferative disorder. Only 5% of cases involve the central nervous system. We describe a 10-year-old girl with pain in her lower limbs and back. Spinal magnetic resonance imaging revealed an intradural extramedullary lesion at T9-T10. We decided on surgical treatment. An anatomic/pathologic examination revealed histiocytic-like cells and extensive fibrosis. Immunohistochemistry revealed positivity for CD68 protein and negativity for CD1a protein. Craniospinal magnetic resonance imaging demonstrated an extra-axial lesion in the right frontal region, a small nodule in the left middle cerebellar peduncle, and another small lesion in the right ventral pons. We performed a complete removal of the frontal lesion. The histologic examination produced results compatible with Rosai-Dorfman disease. Most lesions in intracranial Rosai-Dorfman disease mimic meningioma. The definitive diagnosis relies on pathologic and immunohistochemical characteristics. Surgical removal is generally regarded as the treatment of choice. Disease progression after surgical resection is uncommon. Surgical treatment is not recommended until clear disease progression is detected, or focal disease causes neurologic compression. This disease must be included in the differential diagnosis of lesions that mimic meningioma.
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PMID:Multiple involvement of the central nervous system in Rosai-Dorfman disease. 2219 95

Rosai-Dorfman disease (RDD) is a rare non-Langerhans histiocytosis that usually presents with lymphadenopathy. Although isolated involvement of the CNS was considered to be uncommon, numerous cases have been reported in recent years. For RDD of the CNS, the treatment consists, in general, of surgery. In cases of partial resection or relapse, chemotherapy regimens, corticosteroids, and/or radiotherapy have yielded negative results. The authors describe the case of a 57-year-old man with a history of chronic Q fever who presented with aphasia and partial seizure. Computed tomography of the brain revealed a left frontotemporal lesion that was suggestive of a meningioma. The lesion was partially resected and histopathological evaluation revealed the presence of RDD. Nineteen months later, a Jacksonian seizure prompted MRI evaluation, which disclosed a local recurrence of the tumor. Computed tomography and FDG-PET demonstrated that the RDD involved no other site, but the presence of ileitis, noted on ileoscopy, led to the diagnosis of Crohn disease. Treatment with the purine analog azathioprine was initiated, leading to an objective and sustained response in both the RDD tumor and ileitis over 35 months of follow-up. This case report highlights the potential use of a purine analog in cases of relapsing RDD of the CNS and a possible common defect of macrophage regulation in RDD, Crohn disease, and Q fever.
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PMID:Successful treatment with azathioprine of relapsing Rosai-Dorfman disease of the central nervous system. 2272 85

In lymphoplasmacyte-rich meningioma (LRM) meningothelial whorls are overshadowed by exuberant infiltration by lymphocytes, plasma cells and few histiocytes. Hence, lesions with lymphoplasmacytic proliferation form the histological differentials. We describe the, to the best of our knowledge, first case of LRM with occasiona emperipolesis, creating a diagnostic dilemma with Rosai-Dorfman disease (RDD), around the region of sphenoid wing. LRM was favored due to the presence of epithelial membrane antigen (EMA) and vimentin positive meningothelial whorls, forming approximately 10% of the tumor tissue. Documentation of such cases may help to understand the importance of inflammatory cells and meningothelial whorls, as a manifestation of host response at the leptomeninges.
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PMID:Sphenoid wing lymphoplasmacyte-rich meningioma with occasional emperipolesis closely simulating an intracranial Rosai-Dorfman disease: a diagnostic dilemma. 2321 32

Rosai-Dorfman disease is a benign lymphohistiocytosis that often involve lymph nodes and present as massive lymphadenopathy with sinus histiocytosis. The disease is rarely associated with intracranial involvement. Herein, we report a 33-years-old man with recent onset of unconsciousness. According to his past medical history, he was suffering from frontal headache, ataxia and dizziness with no sensory or motor defect since August 2010. At initial work up, MRI showed infiltrating mass in the left parietal region. Microscopically, the mass consisted of infiltration of abundant lymphoplasma cells, neutrophils and some histiocytes scattered in fibrotic background. Emperipolesis (lymphocytophagocytosis) of histiocytic cells made the diagnosis of Rosai-Dorfman disease. Rosai-Dorfman disease should be added in the list of differential diagnosis for a dural mass mimicking meningioma or cerebral mass mimicking glioma, therefore, immunohistochemical staining for EMA, S100 and CD1a should be performed to rule out the differential diagnosis.
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PMID:Intracranial ROSAI-DORFMAN Disease. 2326 85

Rosai-Dorfman disease (RDD) is a rare but well-recognized idiopathic histioproliferative disease affecting the systemic lymph nodes. It is characterized by an unusual proliferation of histiocytic cells. Intracranial localization is a rare manifestation of RDD. The clinical and radiological differentiation from meningiomas is difficult, and can only be achieved after histological examination. This entity should be considered in the differential diagnosis of dural based lesions mimicking meningioma. We report 2 cases of isolated intracranial RDD. The first patient had a large frontal lesion in addition to smaller multiple intracranial lesions. The second patient had only one parasagittal lesion. The diagnosis was confirmed on histopathological examination after surgical excision. The pertinent literature is also reviewed.
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PMID:Isolated intracranial Rosai-Dorfman disease: report of two cases and a review of the literature. 2410 Dec 72


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