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)

A 57-year-old man presented with a history of nasal obstruction of five to six years duration. 'Nasal polyps' were removed on several occasions. He had previously had an episode of paraplegia which resolved after the removal of a spinal tumour. Histology from both sites was thought to represent a malignant fibrous histiocytoma. On presentation the patient had computed tomographical (CT) evidence of extensive ethmoidal disease, with threatened intracranial extension. He also had evidence of lung and retroperitoneal disease with pancreas and kidney involvement. The ethmoidal disease was considered potentially lethal and therefore a craniofacial resection was performed. Review of all the histology revealed that the diagnosis was extranodal Rosai-Dorfman disease (sinus histiocytosis). The patient's course is described, and the literature on this disease of unknown aetiology is reviewed.
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PMID:Rosai-Dorfman disease of the paranasal sinuses. 816 19

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

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

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

An 18-year-old man presenting with paraplegia due to spinal epidural infiltration at D6-D7 underwent laminectomy and spinal decompression followed by near-complete resolution of neurological symptoms; but this resolution was followed by progressively enlarging cervical lymph nodes. Leucocytosis, an elevated erythrocyte sedimentation rate, a marked polyclonal hypergammaglobulinemia and a moderate hepatosplenomegaly were found. The diagnosis of sinus histiocytosis with massive lymphadenopathy (SHML), also known as Rosai-Dorfman disease was established histologically by lymph node biopsy about 2 years after presentation.
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PMID:A young male with paraplegia, massive cervical adenopathy and nondiagnostic biopsy specimens. 1759 39