Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
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.
 ...
PMID:Rosai-Dorfman disease presenting as a pituitary tumour. 1034 67

Rosai-Dorfman disease (RDD) is a rare idiopathic histiocytic disorder that only occasionally involves the central nervous system (CNS). Previous cases of RDD involving the CNS were generally seen in adults. Pediatric cases of RDD are rare, and the disease in these cases typically has an indolent clinical course. In this report, the authors describe a pediatric case of intracranial RDD with rapid clinical and radiological progression. A previously healthy 13-year-old girl presented with a 15-day history of progressive left-sided headaches, vomiting, and fever. On examination she was pyrexial but otherwise normal. Neuroimaging results demonstrated an extraaxial left frontal lesion with peripheral enhancement. A bur hole was drilled over the lesion to obtain a tissue sample and de-bulk the lesion. The initial histological results showed a nonspecific inflammatory lesion. Postoperatively, the patient was asymptomatic, and neuroimaging results confirmed a significant reduction in the size of the lesion. Repeated neuroimaging 3 months later, however, revealed a large recurrence of the lesion, which was removed macroscopically by a craniotomy. Histological analysis of the tissue confirmed the RDD diagnosis. At the latest follow-up (12 months) the patient had remained asymptomatic with no evidence of recurrence on neuroimaging. This is the first reported case of intracranial RDD with an aggressive clinical course.
 ...
PMID:Cerebral localization of Rosai-Dorfman disease in a child. Case report. 1845 87