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, or Rosai-Dorfman disease (RDD), was first described in 1969 as a reactive condition of unknown etiology that is characterized by a proliferation of histiocytes exhibiting emperipolesis of both lymphocytes and plasma cells. It usually presents with painless cervical lymphadenopathy either with or without extranodal manifestations. Intracranial involvement of this disease is extremely rare. Intracranial RDD occurs in <5% of all patients with extranodal disease. Here, we report a case of RDD with isolated intracranial involvement. A 67-year-old male presented with a long-standing headache, retro-ocular pain, and progressive visual loss of the left eye. Magnetic resonance imaging showed features of optic nerve meningioma. The histopathology revealed sheets of histiocytes displaying emperipolesis. These histiocytes were S100 positive; however, a CD1a and epithelial membrane antigen were negative.
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PMID:A Rare Case of Isolated Intracranial Rosai-Dorfman Disease Mimicking Optic Nerve Meningioma: A Case Report and Literature Review. 3028 62

The association between Rosai-Dorfman Disease (RDD) and cancer was reported for the first time in 1984. However, there are still a small number of reports of this association. We describe a 60-year-old woman who presented with a focal onset motor seizure followed by tonic-clonic generalization and persistent headache. Magnetic Resonance Imaging disclosed an irregular hyperintense lesion in T2 and vasogenic edema in the left parietal region. Immunohistochemical analysis of a biopsy fragment was positive for protein S-100 and CD68 and negative for CD1a, compatible with the diagnosis of RDD. She previously had breast cancer six years earlier and had used tamoxifen for two years and anostrozol for three years after diagnosis of cancer. RDD has been already associated with different cancers, such as Hodgkin's lymphoma, non-Hodgkin's lymphoma, follicular lymphoma, melanoma, adenocarcinoma and small cell lung cancer. As far as we know, this is the first report of an association between breast cancer and RDD.
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PMID:Isolated central nervous system Rosai-Dorfman disease and breast cancer: an unusual presentation. 3029 95

Rosai-Dorfman disease is an uncommon lymphoproliferative disorder, and multiple intracranial involvement in disseminated disease is exceedingly rare. We present a case of a 52 year-old female who presented with intractable headaches and a history of Rosai-Dorfman disease unresponsive to chemo- and radiation therapies. She was found to have new multiple intracranial masses that were treated with surgical excision. Pathology confirmed a diagnosis of intracranial Rosai-Dorfman. The disease presentation, radiographic appearance, histology, treatment, and prognosis are briefly reviewed.
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PMID:Multiple Intracranial Rosai-Dorfman Disease: A Case Report. 3157 81

A 46-year-old male experienced progressive neurocognitive decline, weight loss, intermittent headaches, and weakness over 6 months. Magnetic resonance imaging of the brain revealed hydrocephalus and the spinal cord imaging showed diffuse leptomeningeal enhancement with prominent nerve root involvement. Intradural biopsy of lumbar arachnoid tissue found mixed inflammatory infiltrate consisting predominantly of histiocytes, S100 and CD68 positivity, and lymphocytophagocytosis (emperipolesis) consistent with extranodal Rosai-Dorfman disease. Rosai-Dorfman disease, a non-Langerhans cell histocytic disorder, can mimic the appearance of neurosarcoidosis and leptomeningeal carcinomatosis and should remain on the differential of a patient presenting with diffuse leptomeningeal enhancement, a common occurrence on a neurohospitalist service.
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PMID:Rosai-Dorfman Disease: A Less Common Cause of Leptomeningeal and Nerve Root Enhancement. 3298 53


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