Central Nervous System Rosai-Dorfman-Destombes Disease

Preferred Label : Central Nervous System Rosai-Dorfman-Destombes Disease;

NCIt synonyms : Central Nervous System Rosai-Dorfman Disease;

NCIt definition : Rosai-Dorfman-Destombes disease affecting the central nervous system. It presents with localized or multiple dural masses, parenchymal, and/or intrasellar lesions.;

Neoplastic status : Undetermined;

NCI Metathesaurus CUI : CL1779164;

Details

Origin ID

C186663;

UMLS CUI

C5670693;

Disease may have findings
- Extranodal Disease [NCIt concept]
- Indolent Clinical Course [NCIt concept]
Has associated anatomic sites
- Central Nervous System [NCIt concept]
- Hematopoietic System [NCIt concept]
- Hematopoietic and Lymphatic System [NCIt concept]
- Lymphatic System [NCIt concept]
- Nervous System [NCIt concept]
Semantic type(s)
- Disease or Syndrome [UMLS semantic type]
disease_excludes_finding
- CD1a-Positive Neoplastic Cells Present [NCIt concept]
- CD30-Positive Neoplastic Cells Present [NCIt concept]
- HMB-45-Positive Neoplastic Cells Present [NCIt concept]
disease_excludes_molecular_abnormality
- Clonal Immunoglobulin Gene Rearrangement [NCIt concept]
- Clonal T-Cell Receptor Gene Rearrangement [NCIt concept]
disease_has_abnormal_cell
- Atypical Histiocyte [NCIt concept]
- Neoplastic Cell [NCIt concept]
- Neoplastic Hematopoietic and Lymphoid Cell [NCIt concept]
- Neoplastic Histiocytic and Dendritic Cell [NCIt concept]
disease_has_finding
- CD68-Positive Neoplastic Cells Present [NCIt concept]
- Cervical Lymphadenopathy [NCIt concept]
- Emperipolesis [NCIt concept]
- Fever [NCIt concept]
- Histiocytosis [NCIt concept]
- Massive Sinusoidal Histiocytic Infiltrate [NCIt concept]
- Plasmacytic Infiltrate [NCIt concept]
- Polyclonal Hypergammaglobulinemia [NCIt concept]
- Rare Lesion [NCIt concept]
- S-100-Positive Neoplastic Cells Present [NCIt concept]
disease_has_normal_cell_origin
- Hematopoietic and Lymphoid Cell [NCIt concept]
- Histiocyte [NCIt concept]
- Histiocytic and Dendritic Cell [NCIt concept]
disease_has_normal_tissue_origin
- Hematopoietic Tissue [NCIt concept]
- Hematopoietic and Lymphoid Tissue [NCIt concept]
- Lymphoid Tissue [NCIt concept]
disease_has_primary_anatomic_site
- Hematopoietic and Lymphatic System [NCIt concept]
disease_may_have_molecular_abnormality
- ARAF Gene Mutation [NCIt concept]
- KRAS Gene Mutation [NCIt concept]
- MAP2K1 Gene Mutation [NCIt concept]
- NRAS Gene Mutation [NCIt concept]

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