Disseminated Juvenile Xanthogranuloma

Preferred Label : Disseminated Juvenile Xanthogranuloma;

NCIt synonyms : Disseminated JXG;

NCIt definition : Juvenile xanthogranuloma disseminated to extracutaneous sites including mucosal surfaces, lung, central nervous system, pituitary, lymph nodes, eye, liver, and bone marrow. It is characterized by the presence of lipid-laden, foamy histiocytes and Touton-type giant cells forming nodules in the affected anatomic sites.;

Alternative definition : NICHD: Juvenile xanthogranuloma disseminated to extracutaneous sites, including mucosal surfaces, lung, central nervous system, pituitary, lymph nodes, eye, liver, and bone marrow.;

Neoplastic status : Undetermined;

Détails

Origin ID

C81772;

UMLS CUI

C2825742;

Automatic exact mappings (from CISMeF team)
- Disseminated juvenile xanthogranuloma [ICD-11 More detail]
- Multiple eruptive juvenile xanthogranuloma (disorder) [SNOMED CT concept]
Disease may have findings
- CD68-Positive Neoplastic Cells Present [NCIt concept]
Has associated anatomic sites
- Hematopoietic and Lymphatic System [NCIt concept]
Semantic type(s)
- Neoplastic Process [UMLS semantic type]
concept_is_in_subset
- NCIt Neoplasm Core Terminology [NCIt concept]
- NICHD Terminology [NCIt concept]
- Pediatric Hematology-Oncology Terminology [NCIt concept]
disease_excludes_finding
- 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
- Lipid-Laden Macrophage [NCIt concept]
- Neoplastic Cell [NCIt concept]
- Neoplastic Hematopoietic and Lymphoid Cell [NCIt concept]
- Neoplastic Histiocytic and Dendritic Cell [NCIt concept]
- Touton Giant Cell [NCIt concept]
disease_has_finding
- Extracutaneous Dissemination of Disease [NCIt concept]
- Histiocytosis [NCIt concept]
- Juvenile Lesion [NCIt concept]
- Rare Lesion [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 and Lymphoid Tissue [NCIt concept]
disease_has_primary_anatomic_site
- Hematopoietic and Lymphatic System [NCIt concept]
disease_may_have_associated_disease
- Neurofibromatosis Type 1 [NCIt concept]
disease_may_have_molecular_abnormality
- BRAF Gene Mutation [NCIt concept]

Position du mot-clé dans l'(les) arborescence(s) :

Vous pouvez consulter :

recommendations
documents concerning education
documents for patients