loading...| Preferred Name |
neuroblastoma |
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| Synonyms |
NB neuroblastoma (morphologic abnormality) neuroblastoma NOS (morphologic abnormality) [M]Neuroblastoma NOS (morphologic abnormality) [M]Neuroblastoma NOS Neuroblastomas Central neuroblastoma Neuroblastoma, NOS NB - Neuroblastoma Sympathicoblastoma neuroblastoma (Schwannian Stroma-Poor) (neuroblastoma NOS) or (sympathicoblastoma) neuroblastoma neural Crest tumor, malignant neuroblastoma (Schwannian Stroma-poor) neuroblastoma, malignant |
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| Definitions |
A common neoplasm of early childhood arising from neural crest cells in the sympathetic nervous system, and characterized by diverse clinical behavior, ranging from spontaneous remission to rapid metastatic progression and death. This tumor is the most common intraabdominal malignancy of childhood, but it may also arise from thorax, neck, or rarely occur in the central nervous system. Histologic features include uniform round cells with hyperchromatic nuclei arranged in nests and separated by fibrovascular septa. Neuroblastomas may be associated with the opsoclonus-myoclonus syndrome (MeSH). A neuroblastic tumor characterized by the presence of neuroblastic cells, the absence of ganglion cells, and the absence of a prominent Schwannian stroma formation. A common neoplasm of early childhood arising from neural crest cells in the sympathetic nervous system, and characterized by diverse clinical behavior, ranging from spontaneous remission to rapid metastatic progression and death. This tumor is the most common intraabdominal malignancy of childhood, but it may also arise from thorax, neck, or rarely occur in the central nervous system. Histologic features include uniform round cells with hyperchromatic nuclei arranged in nests and separated by fibrovascular septa. Neuroblastomas may be associated with the opsoclonus-myoclonus syndrome. (From DeVita et al., Cancer: Principles and Practice of Oncology, 5th ed, pp2099-2101; Curr Opin Oncol 1998 Jan;10(1):43-51) Neuroblastoma (NB) is the most common solid, extracranial childhood tumor. It is an aggressive pediatric cancer that originates from neural crest tissues of the sympathetic nervous system. NB is a disease of the sympaticoadrenal lineage of the neural crest, with tumors forming anywhere in the sympathetic nervous system. The tumors most commonly arise in the abdomen (65%), however, they also occur in the neck, chest, and pelvis. Approximately 50% of patients present with evidence of metastasis. Frequent metastasis sites include cortical bone, bone marrow, liver, and lymph nodes. The most common genetic change is MYCN amplification, which occurs in approximately 20% of patients, and is strongly correlated with advanced stage NB. Additionally, deletions of the short arm of chromosome 1 (1p) are found in 25–35% of patients and can be correlated with MYCN amplification. Outside of MYC linked changes, allelic loss of 11q is present in 35–45% of patients and is also associated with high-risk disease features. |
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| ID |
http://www.ebi.ac.uk/efo/EFO_0000621 |
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| comment |
NB is a disease of the sympaticoadrenal lineage of the neural crest, with tumors forming anywhere in the sympathetic nervous system. The tumors most commonly arise in the abdomen (65%), however, they also occur in the neck, chest, and pelvis. Approximately 50% of patients present with evidence of metastasis. Frequent metastasis sites include cortical bone, bone marrow, liver, and lymph nodes. The most common genetic change is MYCN amplification, which occurs in approximately 20% of patients, and is strongly correlated with advanced stage NB. Additionally, deletions of the short arm of chromosome 1 (1p) are found in 25–35% of patients and can be correlated with MYCN amplification. Outside of MYC linked changes, allelic loss of 11q is present in 35–45% of patients and is also associated with high-risk disease features. |
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| closeMatch | ||
| database_cross_reference |
OMIM:613013 OMIM:613014 MeSH:D009447 MedDRA:10029260 SNOMEDCT:87364003 NIFSTD:birnlex_12631 OMIM:256700 SNOMEDCT:432328008 NCIt:C3270 DOID:769 NCIT:C3270 Orphanet:635 SCTID:432328008 UMLS:C0027819 MONDO:0005072 GARD:7185 MESH:D009447 ICDO:9500/3 ONCOTREE:NBL |
|
| definition |
A common neoplasm of early childhood arising from neural crest cells in the sympathetic nervous system, and characterized by diverse clinical behavior, ranging from spontaneous remission to rapid metastatic progression and death. This tumor is the most common intraabdominal malignancy of childhood, but it may also arise from thorax, neck, or rarely occur in the central nervous system. Histologic features include uniform round cells with hyperchromatic nuclei arranged in nests and separated by fibrovascular septa. Neuroblastomas may be associated with the opsoclonus-myoclonus syndrome (MeSH). A neuroblastic tumor characterized by the presence of neuroblastic cells, the absence of ganglion cells, and the absence of a prominent Schwannian stroma formation. A common neoplasm of early childhood arising from neural crest cells in the sympathetic nervous system, and characterized by diverse clinical behavior, ranging from spontaneous remission to rapid metastatic progression and death. This tumor is the most common intraabdominal malignancy of childhood, but it may also arise from thorax, neck, or rarely occur in the central nervous system. Histologic features include uniform round cells with hyperchromatic nuclei arranged in nests and separated by fibrovascular septa. Neuroblastomas may be associated with the opsoclonus-myoclonus syndrome. (From DeVita et al., Cancer: Principles and Practice of Oncology, 5th ed, pp2099-2101; Curr Opin Oncol 1998 Jan;10(1):43-51) Neuroblastoma (NB) is the most common solid, extracranial childhood tumor. It is an aggressive pediatric cancer that originates from neural crest tissues of the sympathetic nervous system. |
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| exactMatch |
http://identifiers.org/mesh/D009447 http://linkedlifedata.com/resource/umls/id/C0027819 http://purl.obolibrary.org/obo/Orphanet_635 http://purl.obolibrary.org/obo/NCIT_C3270 |
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| gwas_trait |
true |
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| has_exact_synonym |
neuroblastoma (morphologic abnormality) neuroblastoma NOS (morphologic abnormality) [M]Neuroblastoma NOS (morphologic abnormality) [M]Neuroblastoma NOS Neuroblastomas Central neuroblastoma Neuroblastoma, NOS NB - Neuroblastoma Sympathicoblastoma neuroblastoma (Schwannian Stroma-Poor) (neuroblastoma NOS) or (sympathicoblastoma) neuroblastoma neural Crest tumor, malignant neuroblastoma (Schwannian Stroma-poor) neuroblastoma, malignant |
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| has_related_synonym |
NB |
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| id |
EFO:0000621 |
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| in_subset |
http://purl.obolibrary.org/obo/mondo#ordo_disease http://purl.obolibrary.org/obo/mondo#rare http://purl.obolibrary.org/obo/mondo#nord_rare |
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| label |
neuroblastoma |
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| notation |
EFO:0000621 |
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| preferred label |
neuroblastoma |
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| prefLabel |
neuroblastoma |
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| see also |
https://rarediseases.info.nih.gov/diseases/7185/neuroblastoma |
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| term editor |
Tomasz Adamusiak James Malone |
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| excluded_subClassOf | ||
| subClassOf |