The causes of most neuroblastomas are unknown. In the fetus, cells called neuroblasts normally develop into nerve cells and tissues. However, if these cells do not become mature and instead continue to grow rapidly as immature cells, they can potentially become cancerous.

The failure of cells to mature and stop growing is caused by DNA mutations (changes). In most cases of neuroblastoma, these mutations occur due to some factor or combination of factors that have not yet been identified. However, there is a familial or inherited form of neuroblastoma that may affect more than one child in a family. 

Usually, neuroblastomas start in a single site. In inherited neuroblastomas, the original tumors are located in several different places in addition to the areas to which they have spread. Inherited neuroblastomas spread more aggressively and affect 1 to 2 percent of all patients, according to the American Cancer Society (ACS).

Research continues to focus on the factors that may influence risks of non-inherited neuroblastoma. Geographic or environmental factors such as pollution or toxins in the environment have not been linked to neuroblastoma, although one study linked codeine use in pregnant or nursing mothers to increased risk. Gestational diabetes has also been linked to neuroblastoma.