neuroblastoma definition

[cmungall]

currently we go with the NCIT:

• def: "A neuroblastic tumor characterized by the presence of neuroblastic cells, the absence of ganglion cells, and the absence of a prominent Schwannian stroma formation." [NCIT:C3270]
• def: "Neuroblastoma is a malignant tumor of neural crest cells, the cells that give rise to the sympathetic nervous system, which is observed in children." [Orphanet:635]

Ideally we would have a citation for this.

• https://www.ncbi.nlm.nih.gov/pubmed/21295685 "Since NB is thought to be an embryonal tumor that is derived from precursor cells of the peripheral (sympathetic) nervous system"

[pnrobinson]

I think both are right, the neuroblastic cells are derived from neural crest (see https://medicine.uiowa.edu/iowaprotocols/neural-crest-cell-tumors-pnt-peripheral-neuroblastic-tumors).

How about def: 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. PMID: 32903387

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. @cmungall @nicolevasilevsky