Neuroblastoma

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Overview

Neuroblastoma, a rare form of paediatric cancer, forms in the immature nerve cells, called neuroblasts. In almost all cases, it occurs in children aged five or below. Also, it can form in babies even before they are born.

Neuroblastomas could be high-grade or low-grade. This condition largely affects the neuroblasts present in the adrenal glands, which are located right above the kidneys. Nevertheless, neuroblastoma can also form in the nerve tissues present in the neck, spinal cord, chest and abdomen region. If not treated on time, neuroblastoma can spread to other organs.

Symptoms

Depending on the location of neuroblastoma, the symptoms may vary, and they may be mild or severe. The common symptoms associated with neuroblastoma include:

Presence of lump in the neck, chest or abdomen
Extreme tiredness
Cough
Fever
Bulging eyes
Loss of appetite
Digestion problems – pain, bloating, constipation and diarrhoea
Pale skin caused due to anaemia
Mobility issues
Increased blood pressure
Horner’s syndrome – characterised by the tiny pupils, droopy eyelid and sweating seen only on face’s one side
Bone pain
Hampered balance – poor coordination and movements
Uncontrollable eye movements
Breathing difficulties

Courses

There are no known causes for neuroblastomas. It is not associated with any environmental or lifestyle risk factors either. The risk factors identified for this condition are unchangeable.

Family History: Those having a family history of this disease have an increased risk of developing it.
Birth Defects: Babies born with specific birth defects are also at an increased risk of developing neuroblastoma.

Diagnosis

As mentioned earlier, neuroblastoma diagnosis happens before age 5 in most cases. In some cases, it can be diagnosed in foetuses during the regular pregnancy ultrasound scanning as well. Following are the test methods used for the detection and diagnosis of neuroblastoma:

a. Physical Exam: Initially, the doctor physically examines the patient for the signs of neuroblastoma. It will be a neurological exam, wherein the doctor tests the patient’s nerve functions, coordination and reflexes. If the doctor suspects neuroblastoma, then he/she may recommend additional tests.

b. Blood and Urine Tests: Blood tests and urine tests may be recommended to check for the levels for specific metabolites that could indicate the presence of cancer. They are also important to assess liver function, kidney function, electrolyte balance, etc.

c. Imaging Tests: Multiple imaging tests, such as X-ray scan, ultrasound, MRI scan and CT scan could be suggested by the doctor to obtain more details about the disease, such as the exact location, the organs that it has spread to, etc.

Along with these, there is a very specific imaging test for neuroblastoma called the Methyl Iodo Benzyl Guanidine (MIBG) scan. It employs the radioactive compound, 123-iodinated MIBG radiotracer, which is only absorbed by neuroblastoma cells. This radiotracer is given intravenously to the patient, and the next day, a special scanner is used to capture the images of the patient’s internal organs, where the clusters of cancer cells appear as visible spots. This is a safe procedure.

In a few cases, a PET scan may also be recommended, especially in the cases where the neuroblastoma tumours fail to absorb MIBG radiotracer.

d. Biopsy: Biopsy involves collecting a small amount of tissue from the suspected area and sending it for analysis. This sample is examined under a microscope for the presence of neuroblastoma cells. It could also be used to look out for specific chromosome alterations that could be associated with neuroblastoma. Additionally, the doctor may also recommend a bone marrow biopsy, wherein the bone marrow is collected and examined for signs of neuroblastoma.

Treatment

Upon considering a myriad of factors, the doctor may recommend a treatment plan that could involve the following treatment approaches:

a. Surgery: Surgery is often combined with chemotherapy while treating neuroblastoma. This helps in shrinking the tumour before the surgery and eliminating the residual cancer cells after the surgery and also bringing down the risk of relapse later. Surgery aims to remove the tumour; however, removal of the entire tumour may not be possible in all cases.

b. Chemotherapy: Chemotherapy uses strong anticancer drugs to stop the neuroblastoma cells from dividing and spreading to other organs. Chemotherapy for neuroblastoma can last for weeks to months, depending on the severity of the condition.

c. Stem Cell Transplant: High-dose chemotherapy destroys bone marrow cells that are responsible for the formation of immune cells. Therefore, the doctor may recommend stem cell transplant after chemotherapy in a few cases depending on the intensity of the chemotherapy treatment. Before chemotherapy, the stem cells from the patient’s bone marrow are collected and stored in a freezer, and these cells are injected back into the body, where they start rebuilding the patient’s immune system.

d. Radiation Therapy: Radiation therapy is a localised therapy that kills cancer cells using powerful radiation beams. In neuroblastoma patients, radiation therapy is used to reduce the risk of a relapse. It is largely recommended for those with high-risk neuroblastoma.

e. Iodine 131-MIBG Therapy: During this procedure, the radioactive combination – iodine 131-MIBG is injected into the body, and the radiation emitted by it destroys the tumour cells. This therapy is not widely used for neuroblastoma.

f. Immunotherapy: Immunotherapy is another treatment approach that is not widely used. This treatment triggers the body’s immune system to launch an attack against the cancer cells. Immunotherapy may be recommended for neuroblastomas that have shown no response to chemotherapy and radiation therapy.

Frequently Asked Questions

1. Is neuroblastoma treatable?

Yes, neuroblastoma is treatable.

It is more common among children, and therefore, parents must pay attention to the symptoms that are associated with this disease. However, the parents need not worry as there are plenty of treatment options available for the successful management of neuroblastoma.

2. Is early detection of neuroblastoma possible?

Yes, early detection of neuroblastoma is possible. In some cases, it can be detected in the foetuses during the regular ultrasound scans that happen during the pregnancy.

In other cases, neuroblastomas may be diagnosed when the child visits the doctor for other health problems or regular health check-ups.

Neuroblastomas cause various symptoms, and the parents must be careful enough to identify anything that is not normal and report it to the doctor immediately – this is important for early diagnosis and timely treatment.

3. Does neuroblastoma come back?

Yes, neuroblastoma can come back in a few cases. However, if they are detected early, they can be treated successfully.

Parents of the patients need to ensure that no follow-up appointment is missed and the patients are given medications and other necessary follow-up support without fail.

4. Can neuroblastoma be prevented?

Neuroblastoma cannot be prevented as its causes are not known, and it does not have any lifestyle-based risk factors. If one of the parents has been diagnosed with neuroblastoma or if the baby is born with congenital disorders, extra care should be taken. Parents can even talk to the doctor to understand the best practices that will help in reducing neuroblastoma risk for their children.