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)

Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) is a nonneoplastic lymphoproliferative disorder that usually presents with massive painless cervical lymphadenopathy accompanied by fever and weight loss. Extranodal involvement occurs in over 40% of patients; however, isolated extranodal disease without lymph node involvement is unusual. Extranodal involvement of the nervous system is unusual and isolated central nervous system disease in the absence of nodal disease is distinctly rare. The author describes the case of a 35-year-old man who presented with paraplegia and an enhancing intramedullary lesion on magnetic resonance imaging that was suggestive of a primary spinal cord tumor. The lesion was completely resected and histologically proved to be consistent with an isolated extranodal case of Rosai-Dorfman disease. Although a number of cases of this entity involving the CNS have been published, the author believes this case represents the first report of intramedullary spinal cord involvement as the initial and only sign of disease.
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PMID:Isolated extranodal sinus histiocytosis presenting as an intramedullary spinal cord tumor with paraplegia. Case report. 881 78

A case of cutaneous Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) manifestating as a recurrent breast tumor is reported. The tumor occurred on the left breast of a 35-year-old woman. Before arriving at the correct diagnosis, four biopsies had been performed with various diagnoses of chronic inflammation, plasma cell mastitis and inflammatory pseudotumor. Numerous typical histiocytes with lymphophagocytosis appeared in the final excised specimen, and a correct diagnosis was made. Ultrastructural examination revealed no evidence of Birbeck granule. The literature concerning Rosai-Dorfman disease manifestating as breast tumor is reviewed. Since the diagnosis is often overlooked in the absence of lymphadenopathy, a high index of suspicion is required to recognize this rare cutaneous Rosai-Dorfman disease.
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PMID:Cutaneous Rosai-Dorfman disease manifestating as recurrent breast tumor: a case report. 921 25

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

We report the case of a woman who developed an early relapse of a squamous cell carcinoma (SCC) and was thus restaged twice within a year using [(18)F]fluorodeoxyglucose (FDG) positron emission tomography (PET). While there was no evidence of metastatic tumor outspread, focally increased FDG uptake was visible in numerous nodes but showed no change during the period between the two PET scans. These nodes, predominantly located at the proximal extremities, ranged in size from about 1 cm to over 6 cm. They were located subcutaneously, showed a red/bluish livid color and were of stout consistency. These nodes occurred first after radiochemotherapy for a non-Hodgkin's lymphoma (NHL) about 6 years earlier and slowly increased in size and number. One node of the right forearm was resected and ex-vivo beta-imaging, directly measuring the positron emission of the intranodal FDG distribution, was done and showed an overall increased glucose utilization with distinct spots of high metabolism. Histopathological work-up of the tumor showed widespread granulomatous tissue with lymphocyte follicles. Immunostaining showed the tumor to be positive for S100, CD68 and vimentin. Rosai-Dorfman disease (RDD) was diagnosed and no evidence of a potential relapse of the previous NHL was detected. RDD is a rare disease that is associated with the multifocal growth of benign tumors. The lesions are metabolically highly active. The correlation of the beta-imaging and histopathological results showed a high metabolism within granulomatous tissue with more intense metabolism within lymphocyte follicles.
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PMID:PET imaging of Rosai-Dorfman disease: correlation with histopathology and ex-vivo beta-imaging. 1456 72

Rosai-Dorfman disease is a rare, non-neoplastic disease characterized by an unusual proliferation of histiocytic cells. It rarely manifests intracranially, and only 50 cases of intracranial lesions have been reported. We describe an unusual case of a huge, solid mass in the paranasal sinuses, orbits, cavernous sinuses, and suprasellar cisterns. A 29-year-old patient was admitted to our hospital with nasal obstruction and proptosis and visual loss in the right eye. A biopsy was done at another institution and the diagnosis was "pseudotumor of orbit". A right-sided cranio-orbitozygomatic craniotomy combined with a right-sided lateral rhinotomy was used to excise the tumor with right orbital exenteration. The histopathological diagnosis was consistent with Rosai-Dorfman disease. The patient underwent postoperative chemotherapy. Involvement of the central nervous system in Rosai-Dorfman disease is rare, but the disease's ability to mimic other pathologies underlines its importance.
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PMID:Giant intracranial Rosai-Dorfman disease. 1517 15

We report the clinical and pathological findings of a 19-year-old Venezuelan patient with a proximal tibial lesion showing the histological and immunohistochemical characteristics of Rosai-Dorfman disease. The radiological studies showed a lithic bone lesion that was interpreted as a giant cell tumor.
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PMID:Extranodal Rosai-Dorfman disease. Clinical and pathological characteristics in a patient with a pseudotumor of bone. 1523 51

There have been no reports on choromosomal aberrations of benign bone tumors revealed by comparative genomic hybridization (CGH). CGH analysis of benign tumors may be useful in understanding the mechanism of tumorigenesis with comparisons to malignant tumors. There were 4 tumors (2 enchondromas, one chondromyxoid fibroma, and one osteoid osteoma) and 8 tumor-like conditions (4 aneurysmal bone cysts (ABCs), one eosinophilic granuloma, one fibrous dysplasia, one solitary bone cyst, and one Rosai-Dorfman disease) available for analysis. One of 2 enchondromas and one of 4 ABCs exhibited rapid growth. Six lesions showed chromosomal aberrations, while 6 others did not. The most frequent aberrations were the loss of a whole chromosome-19 in 6 cases, the loss of chromosome-arm 22q in 4 cases, and the loss of chromosome-arm 17p in 3 cases. Gains were seen in 13q21 in 2 cartilaginous tumors and at 12q15-q21 in eosinophilic granulomas. Therefore, in benign bone tumors or tumor-like lesions, chromosomal aberrations are not frequent; however, some clear tendencies of clustering of aberrations can be observed.
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PMID:Genetic imbalances in benign bone tumors revealed by comparative genomic hybridization. 1564 Sep 55

Sinus histiocytosis with massive lymphadenopathy is a benign lymphoproliferative disorder growing in frequency as awareness of the disease increases. Also known as Rosai-Dorfman disease, it typically presents as painless cervical lymphadenopathy with fever and malaise. A review of the literature reveals approximately 400 cases of extranodal involvement and approximately 44 cases of central nervous system involvement. Less than 10 of the reported central nervous system tumors have presented as an epidural spinal cord tumor. The authors describe the case of a 29-year-old woman with progressive paraplegia and leg pain. Magnetic resonance imaging of the thoracic spine revealed a tumor mass from T5 to T9. The tumor was resected, and the spinal column was stabilized with pedicle screw fixation and fusion. Postoperative treatment was initiated with radiation and physical rehabilitation. The following is a case report with literature review of the entity.
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PMID:Atypical presentation of sinus histiocytosis with massive lymphadenopathy as an epidural spinal cord tumor: a case presentation and literature review. 1580 Apr 41


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