Neuroblastoma is a rare type of childhood cancer that develops from immature nerve cells called neuroblasts. It most commonly originates in the adrenal glands, which sit atop the kidneys, but can also begin in nerve tissue in the neck, chest, abdomen, or pelvis. This cancer primarily affects infants and very young children, with most cases diagnosed before the age of five. The behavior of neuroblastoma is highly variable; in some infants, the tumor may disappear on its own, while in other children, it can be an aggressive disease that spreads quickly to other parts of the body. Understanding the mortality rate is crucial for families and healthcare providers, as it helps frame the prognosis and guide complex treatment decisions.
When discussing outcomes for neuroblastoma, medical professionals typically refer to survival rates rather than mortality rates. The most common statistic used is the 5-year relative survival rate, which represents the percentage of children with neuroblastoma who are still alive at least five years after their diagnosis, compared to children in the general population. It is important to remember that this is an average based on data from large groups of patients and cannot predict what will happen in any individual child's case. The prognosis for neuroblastoma is not a single number; instead, it is highly dependent on a classification system that categorizes patients into risk groups. This stratification is critical and is based on several key factors:
- Age at diagnosis, as infants under 18 months generally have a better outlook.
- The stage of the cancer, which describes how much the tumor has grown and whether it has spread (metastasized) to distant parts of the body like the bone marrow, bones, liver, or lymph nodes.
- The tumor’s biology, including specific genetic features like the amplification of the MYCN gene, which is associated with more aggressive cancer growth.
The 5-year survival rates for neuroblastoma vary dramatically across the different risk groups. For children in the low-risk group—typically those who are very young with small, localized tumors and favorable biology—the prognosis is excellent, with a survival rate of over 95%. Treatment for this group may be less intensive and can sometimes involve surgery alone or even careful observation. Children in the intermediate-risk group have survival rates ranging from 90% to 95%; these patients usually require more treatment, including chemotherapy and surgery, but still have a very good prognosis. The greatest challenge lies with the high-risk group, which includes most older children, those with widespread metastatic disease, or those whose tumors have unfavorable biological markers like MYCN amplification. Despite receiving the most intensive and multi-faceted treatments—often involving high-dose chemotherapy, surgery, radiation therapy, stem cell transplant, and immunotherapy—the 5-year survival rate for high-risk neuroblastoma is approximately 50% to 60%. This means that sadly, the mortality rate in this specific group remains high. It is this high-risk category where clinical trials and research are most intensely focused, continually seeking new therapies to improve these odds and offer more hope to families.
What is the life expectancy of an adult with neuroblastoma?
While neuroblastoma is rare in adults, making life expectancy data less extensive than for children, the disease often follows a more indolent or slow-growing course. Despite this, the overall prognosis is generally worse for adults, often due to a lower tolerance for the aggressive treatments used in pediatric patients and the presence of other health conditions. Data from the SEER registry indicates a 5-year disease-specific survival rate of approximately 33% for adults aged 18-60. However, prognosis is highly dependent on the stage of the cancer at diagnosis; one retrospective review found that while adults with localized (Stage I-II) disease had a 5-year survival rate of 83%, this rate dropped to 28% for those with advanced (Stage III-IV) disease.
Can you beat stage 4 neuroblastoma?
While stage 4, high-risk neuroblastoma is an aggressive and historically difficult cancer to treat, significant advancements are making survival increasingly possible. Today, oncologists utilize a multi-pronged attack that combines intensive chemotherapy, surgery, radiation, and immunotherapy. A major breakthrough is the recent FDA approval of difluoromethylornithine (DFMO), marketed as IWILFIN, a maintenance therapy specifically designed to prevent relapse by targeting resilient cancer stem cells after initial treatments are completed. Success stories, like that of Will Lacey who is now a healthy college student after facing a relapse, provide powerful evidence that with these innovative strategies, beating even this aggressive form of neuroblastoma is an achievable reality for more children.
How fast does neuroblastoma progress?
The progression speed of neuroblastoma is highly variable and depends on the specific biological characteristics of the tumor. Some neuroblastomas, particularly in infants, may grow very slowly or even spontaneously regress without any treatment. In contrast, other forms of the disease, especially those classified as high-risk, are highly aggressive and can progress rapidly, often spreading to other parts of the body by the time of diagnosis. Key genetic factors, such as the amplification of the MYCN oncogene, are strongly linked to this rapid progression and a poorer prognosis. Therefore, the behavior of neuroblastoma can range from a slow-growing, localized mass to a quickly metastasizing cancer.
How rare is neuroblastoma in adults?
While neuroblastoma is the most common extracranial solid tumor in children, its diagnosis in adults is exceedingly rare. The estimated incidence is only about one case per 10 million adults per year. Overall, adult-onset neuroblastomas are thought to account for less than 5% of all cases, as the vast majority of patients—over 90%—are diagnosed before the age of five. To illustrate this rarity, one analysis of the Surveillance, Epidemiology, and End Results (SEER) database found that patients aged 20 and older represented just 6.1% of all neuroblastoma cases diagnosed over a nearly 30-year period.
Where does neuroblastoma metastasize to?
When neuroblastoma spreads, or metastasizes, it travels from the original tumor site to distant parts of the body. In infants and children, neuroblastoma most frequently metastasizes to the lymph nodes, bones, bone marrow, liver, and skin. A special metastatic category, known as Stage MS, affects children younger than 18 months and is typically confined to the skin, liver, and/or bone marrow. Although less common, the cancer can also spread to the lungs and brain, which is seen more often in adolescent patients. The extent of this spread is a critical factor that doctors use to determine the cancer's stage and the most appropriate treatment plan.
