Everything you need to know about neuroblastoma in children

What is neuroblastoma and why does it happen?

Neuroblastoma is a cancerous tumor that starts in the peripheral nervous system.
Despite the fact that some neuroblastomas may be related to genetic mutations and seen more in certain families, and despite them sometimes having an association with different syndromes such as Hirschsprung, Neurofibromatosis type I and other syndromes, most neuroblastomas occur without a clear reason.

How common is neuroblastoma in children and at what age does it usually present?

It is the second most common cancer after brain tumors in children. Additionally, it is the most common cancerous tumor in infancy.
Neuroblastomas represent 8-10% of the cancer cases in children, and the median age for presentation is 22 months. About 90% of the cases are diagnosed under the age of 5.

What are the signs and symptoms of neuroblastoma?

Since this is a growth that starts in the peripheral nervous system, which can be found almost anywhere in the body, the signs and symptoms express the primary location of the tumor, its size and its proximity to other systems as well as the extent of the disease. Some of the known symptoms are associated with metastasis and occur more commonly in children above the age of 1.
Sometimes, it is a non-symptomatic tumor that can be found in areas such as the neck, chest or abdomen and sometimes there is pain associated with the location of the tumor, such as abdominal pain, back or limb pain, intestinal obstruction, limp etc.
Sometimes, there are unexplained fever, irritability, failure to thrive and loss of weight, bone pain and blueish lesions around the eyes (racoon eyes).
An additional, different presentation may be related to an autoimmune disorder (paraneoplastic syndrome) and is referred to as opsoclonus-myoclonus-ataxia, characterized by rapid eye movements and instability.

How do doctors diagnose neuroblastoma

The diagnosis is performed with the help of different investigations, depending on the signs and symptoms and the size of the tumor.
The medical workup includes a physical examination, blood tests, different imaging modalities such as CT or MRI and sometimes biopsies and bone marrow examination. An additional investigation that is sometimes done to look for the spread of disease is an MIBG scan – a substance that accumulates in cancerous cells.

Are there special blood tests for neuroblastoma?

Yes.
There are two tumor markers: HVA and VMA that are secreted in the urine and found to be very high in children with neuroblastoma.

How is the severity of the disease established at time of diagnosis?

Depending on the location of the tumor and the extent of spread at the time of diagnosis.
As you will see below, the different severities of the disease affect the management, and it may range from no need for any treatment at all where the child can be simply monitored (a specific type of neuroblastoma that is usually found in young babies and it resolves spontaneously without treatment), to severe disease requiring intensive, individualized treatment with chemotherapy, radiation, surgeries and sometimes bone marrow transplantation.

What are the different therapies available for neuroblastoma in children?

Treatment of this disease has undergone a positive change in the past decades, based on two main understandings
a. Less intensive treatment for a localized disease with low risk
b. Aggressive treatment and new therapies for high risk disease
It is easy to understand that the chosen therapy for neuroblastoma may change depending on the severity of the illness and the patient’s state. When the disease is mild, in young babies with a specific type of disease, sometimes simple monitoring is sufficient to allow for the tumor to resolve spontaneously.
Other mild type of disease may require surgery for resection of the tumor.
If the disease is moderate or severe, there is a combination of different therapies available including surgery for rection of the tumor, chemotherapy, radiation and even bone marrow transplant.
In fact, the treatment is individualized to the patient depending on the characteristics of the disease and the patient’s overall health status.

What are the new therapies available for neuroblastoma?

Fortunately, several therapies have recently been added to the oncologist’s toolbox for treatment neuroblastoma, and these include new therapies such as immune therapy and biological treatments that aim to improve the chances of recovery and reduce side effects. The therapy targets a specific area in the body and is highly expressed in the tumour cells.

Immune therapy as treatment for cancer?

Yes.
This therapy includes specific antibodies that assist the immune system in destroying cells that produce a certain protein, in this case it is a protein that is expressed in the neuroblastoma cells at a higher level.

What about providing support for families of children with neuroblastoma

This is just as important as treating the child.
Families with children who have neuroblastoma, similarly to any other oncological disease, need to receive a lot of support from the medical and para-medical teams.
The emotional support throughout the journey is very important, and a full team of professionals from different fields is needed help these families get through this difficult time.

What does the future hold?

Science never takes a break, and it is always trying to find solutions and therapies to the different stages of the disease. The awareness for neuroblastoma has been increasing in the past few years, and so are the knowledge and the therapies available. There is space for optimism and the hope for a significant improvement in the survival rates and the quality of life of the patients.
There is no doubt that when a child is diagnosed with neuroblastoma the family goes through a difficult and challenging journey. Dealing with the disease requires both medical therapy as well as emotional support. The medical team, together with the valuable teams available at hospitals, provides a support system that helps families cope with the challenges.
I truly believe that the love and strength that the families carry is an inseparable part of the journey and provide the child with the power to fight and overcome difficulties. The familial and professional support is critical for these types of journeys and allows children to cope with the disease in an optimal way and to aspire for a better future.
In summary, neuroblastoma is a cancerous tumor that starts in the peripheral nervous system, and it is the most common cancer in children under 2. The signs and symptoms vary depending on the location of the tumor and the diagnosis is performed through different investigations.
The treatment is derived from many different variables and that is why it is important to be under the care of physicians at hospitals that are aware of the different protocols and the new therapies available for children.