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Query: UMLS:C0019625 (Rosai-Dorfman disease)
 763 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 45-year-old woman had pyrexia, headaches, collapse and hyponatraemia. Intracerebral abscess, bacterial meningitis and subarachnoid haemorrhage were excluded. She was given intravenous antibiotics and gradually recovered. One month later she was readmitted with diplopia, headache and vomiting. Serum sodium was low (107 mmol/l) and a diagnosis of inappropriate ADH secretion was made. MRI scan showed a suprasellar tumour arising from the posterior pituitary gland. A skin rash gradually faded. Serum cortisol, prolactin, gonadotrophins and thyroid hormone levels were low. A pituitary tumour was removed trans-sphenoidally, she had external pituitary radiotherapy, and replacement hydrocortisone and thyroxine. She was well for 12 months when she developed progressive weakness and numbness of both legs. Examination suggested spinal cord compression at the level of T2 where MRI scanning showed an intradural enhancing mass. This spinal tumour was removed and her neurological symptoms disappeared. Nine months after this she developed facial pain and nasal obstruction. CT scan showed tumour growth into the sphenoid sinus and nasal cavities. A right Cauldwell-Luc operation was done and residual tumour in the nasal passages was treated by fractionated external radiotherapy and Prednisolone. Histological examination of the specimens from pituitary, spinal mass, and nasal sinuses showed Rosai-Dorfman disease, a rare entity characterized by histiocytic proliferation, emperipolesis (lymphophagocytosis) and lymphadenopathy. Aged 48 she developed cranial diabetes insipidus. Although Rosai-Dorfman syndrome is rare, it is being reported with increasing frequency, and should be borne in mind as a possible cause of a pituitary tumour.
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PMID:Rosai-Dorfman disease presenting as a pituitary tumour. 1034 67

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

We experienced a rare case of sinus histiocytosis with massive lymphoadenopathy (Rosai-Dorfman disease) accompanying hypertrophic cranial pachymeningitis. The patient is a 64-year-old woman with an early symptom of rt. peripheral facial palsy. She had a 4-month history of headache with a 5-week history of numbness in the rt. supraorbital nerve area, and lost her weight by 10 kg in 2 months. She developed rt. trochlea nerve palsy and numbness in the lt. mandibular nerve area. Laboratory findings showed that ALP, LDH and CRP were higher than normal. Of CT, MRI and MRA, the images of her head were normal. However, the Gd-enhanced MRI only showed a diffuse pachymeningeal enhancement. After about 3 months from the onset of rt. peripheral facial palsy, she died of DIC of unknown etiology. As a result of examinations in anatomical pathology, she was diagnosed as having sinus histiocytosis with massive lymphoadenopathy (Rosai-Dorfman disease). There were a large number of histiocytes on the pachymeninx. These findings suggest that hypertrophic cranial pachymeningitis caused multiple cranial neuropathy. We emphasize that use of Gd-enhanced MRI in the early stage is important for diagnosis.
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PMID:[Rosai-Dorfman disease accompanying hypertrophic cranial pachymeningitis with an early symptom of right peripheral facial palsy]. 1242 22

Intracranial Rosai-Dorfman disease is quite rare. Here, we report a 67-year-old man with a 90 day history of pain and numbness in his right limbs. The patient was suspected of suffering from melanoma. Then he received craniotomy and was finally diagnosed with intracranial Rosai-Dorfman disease. MEDLINE was used to search the related literature; and the diagnosis, mechanism, treatment and prognosis of this rare tumor are discussed.
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PMID:Intracranial Rosai-Dorfman disease mimicking melanoma: a case report and review of the literature. 3266 85