Neuroblastoma is a type of cancer that develops from immature nerve cells, called neuroblasts, which are part of the sympathetic nervous system. It is the most common cancer diagnosed in infants and primarily affects children under the age of five, with the average age of diagnosis being around 17 months. These tumors most often begin in the adrenal glands located on top of the kidneys but can also form in the neck, chest, or pelvis. While many children with neuroblastoma have a good outlook, the disease is responsible for about 15% of all childhood cancer deaths, making it crucial to understand the factors that predict a child's outcome. A key measure used by doctors to understand this prognosis is the 5-year survival rate, which represents the percentage of children who are still alive at least five years after their diagnosis.
The survival rate for a child with neuroblastoma is not a single number; it largely depends on their assigned risk group. Doctors use these groups—low, intermediate, and high—to determine the intensity of treatment needed and to predict the likelihood of a cure. A child's risk group is determined by a complex combination of factors, including their age at diagnosis, the stage of the cancer (how far it has spread), how the tumor cells look under a microscope, and specific biological features of the tumor. These biological markers include the presence of genetic mutations like MYCN amplification and whether the cancer cells have extra chromosomes (known as tumor ploidy). According to the Children’s Oncology Group (COG), the 5-year survival rates vary significantly based on these categories:
- Low-risk group: Higher than 95%
- Intermediate-risk group: Between 90% and 95%
- High-risk group: Approximately 50%
Age is one of the most important predictors of outcome. Younger children generally have a much better prognosis, with the 5-year survival rate for infants diagnosed before their first birthday being as high as 95%. While it was traditionally thought that being under 12 months was the key cutoff, recent studies suggest that being under 18 months may be a more relevant predictor, as older children have a higher chance of relapse. In contrast to the relatively high survival rates in children, neuroblastoma is extremely rare in adults and has a significantly poorer outlook. Overall, the National Cancer Institute (NCI) reports a 5-year survival rate of 68% for all children under 15 with neuroblastoma. It is important to remember that these statistics are based on data from several years ago. Survival rates are continually improving as treatments advance. For example, recent clinical trials combining chemotherapy with new immunotherapy drugs have shown great promise, significantly increasing survival rates for children in the challenging high-risk group.
Can a child recover from stage 4 neuroblastoma?
Yes, a child can recover from stage 4 neuroblastoma, which is also known as Stage M disease under the current staging system. Although this advanced stage is categorized as high-risk and presents significant challenges, recovery is possible for many children. The 5-year overall survival rate for high-risk neuroblastoma is approximately 50-60%, a figure that has improved over time due to therapeutic advances. This recovery is typically achieved through an intensive, multimodal treatment plan that combines several approaches, such as chemotherapy, surgery, radiation, stem cell transplantation, and newer immunotherapies that help the body’s own immune system fight the cancer.
What is the most common age for neuroblastoma?
Neuroblastoma is overwhelmingly a cancer of early childhood, primarily affecting infants and toddlers. The median age at diagnosis is just 17 months, with the average age cited as being between one and two years old. The vast majority of cases—approximately 90%—are diagnosed in children younger than five years of age. A significant number of these diagnoses, about 37%, occur in infants under the age of one. Consequently, it is quite rare for neuroblastoma to be diagnosed in children older than 10 years.
What is the main cause of neuroblastoma?
The exact cause of most neuroblastomas remains unclear. The cancer develops from neuroblasts, which are immature nerve cells found in a developing fetus. Neuroblastoma occurs when these neuroblasts undergo genetic mutations, or changes in their DNA, that prevent them from maturing into functional nerve cells. Instead, these changes instruct the cells to grow and multiply uncontrollably, forming a tumor. While the specific trigger for these mutations is usually unknown, a small percentage of cases, about 1-2%, are hereditary and can be linked to an inherited genetic alteration, most commonly in the ALK gene.
Has anyone survived neuroblastoma?
Yes, many individuals diagnosed with neuroblastoma survive, and survival rates have improved substantially over the last few decades. For children in the high-risk group, the five-year survival rate has dramatically increased from 6% in the early 1990s to 43% in recent years, thanks to advances like high-dose chemotherapy and immunotherapy. While outcomes are often more challenging for adults, survival is still achieved, with large database reviews reporting five-year survival rates around 36% to 46%. Even in cases of relapse, which are very difficult to treat, studies show that a small percentage of patients—around 8-12%—can survive long-term, demonstrating that survival is possible across different stages and patient populations.
How aggressive is neuroblastoma?
The aggressiveness of neuroblastoma varies significantly from one child to another, and its behavior can be unpredictable. To account for this, doctors classify the cancer into different risk groups—low, intermediate, and high—based on factors like the child's age, tumor stage, and specific genetic features. Some low-risk tumors are much less aggressive and may only require surgery or even just careful observation to see if they resolve on their own. In contrast, high-risk neuroblastoma is an aggressive disease that often spreads (metastasizes) to distant parts of the body and requires intensive, complex treatment involving a combination of therapies like chemotherapy, surgery, radiation, and immunotherapy. This risk classification is essential for determining the most appropriate and effective treatment plan for each child.
How long can neuroblastoma go undetected?
There is no specific timeline for how long a neuroblastoma can go undetected, as detection often depends on when and if symptoms develop. In some instances, a tumor can be present before birth and is only discovered by chance on a prenatal ultrasound or during a routine medical checkup in an otherwise healthy-seeming child. However, it most commonly remains undetected until the tumor grows or spreads, leading to noticeable signs like a swollen abdomen, unexplained fevers, bone pain, or bruising around the eyes. Interestingly, some neuroblastomas may go undetected permanently, as studies suggest certain tumors can mature into non-cancerous tissue or even disappear on their own without ever causing health problems.
What is the cause of death in neuroblastoma?
Death from neuroblastoma is primarily caused by complications arising from progressive, treatment-resistant disease and its spread to other parts of the body (metastasis). The growth of tumors can directly interfere with vital functions; for example, paraspinal tumors can compress the spinal cord, and massive liver metastases may cause respiratory compromise in infants. Widespread metastasis is a major factor, with spread to the bone marrow leading to pancytopenia (severe reduction in blood cells), which can result in fatal infections or bleeding. Ultimately, mortality is highest in patients with high-risk disease, where the cancer’s rapid and relentless progression overwhelms the body’s systems despite intensive treatment.
