Guest Author - Adelle Ottavini
Neuroblastoma occurs most commonly in children younger than age 6, and more so in children/babies under the age of 2. The causes of neuroblastoma is still largely unknown, but is recently genetically linked to the ALK gene, very young infectious disease and even exposure by parents to chemicals e.g. hair dye, alcohol, tobacco, and environmental chemicals - at the time of the child’s conception!
This form of cancer is a neuroendocrine tumour in the sympathetic nervous system chain. Big words – I know. The SNS chain is the nerve tissue running from the brain, down the spine. A tumour normally forms in the adrenal glands in the abdomen, just above the kidneys. Tumours can also form in the neck, chest, abdomen and pelvis. The child’s symptoms will largely depend on the region of the tumour. In the adrenal glands or abdomen (tummy) – symptoms will be bloating, constipation and/or difficulty in passing urine. A neck tumour will be noticed as a bump/lump in the neck, and can also cause difficulty in swallowing. In the chest – difficulty in breathing and/or swallowing will be experienced. If the tumour is close to the spinal cord, weakness in standing, walking and crawling will be most evident. In some cases blue coloured lumps can be observed on the child’s skin. Fever, fatigue and appetite loss, pain in bones and jerky eye and/or muscle movements are also symptoms of neuroblastoma. Unfortunately one will often find that the cancer has metastasised by the time discovery is made.
Diagnosing your child will entail – blood tests, x-rays, CT and MRI scans, bone marrow tests and mIBG scans, and urine tests. The urine test will also be a ‘tumour marker’ in future. Children with neuroblastoma have vanillylmandelic acid or homovanillic acid present in their urine. When the level of these acids in the urine falls, it indicates that the treatment is working! mIBG scans will show metastasising and positioning of tumours.
Staging of neuroblastoma is quite simple. There are three ‘steps’ – low risk (highly curable), medium risk (therapy needed) and high risk (difficult treating). Oncologists will explain each stage better, but here is an outline:
Stage 1: Tumour is in one area only and can be removed surgically.
Stage 2A: Tumour is in one area only but can’t be completely removed.
Stage 2B: Tumour is in one area only but has spread to local (nearby) lymph nodes.
Stage 3: Cancer has spread to surrounding organs but not to distant (further away) organs.
Stage 4: Cancer has spread to distant organs e.g. lymph nodes, bone marrow, liver, bone or skin.
Stage 4S: Tumour is local (1, 2A, 2B) but cancer is also found in liver, skin and/or bone marrow. Stage 4S occurs more so in babies under 12months.
Relapsed neuroblastoma usually has a poor treatable outcome.
Stage 1 and 2A only entails surgery and observation (very very important). From stage 2B, surgery and chemotherapy will be performed. Stages 3 and 4 will require surgery (if possible), intensive chemotherapy, radiation therapy and even bone marrow transplant will be considered.
Side effects of these therapies are: tiredness, nausea, diarrhoea, bruising and bleeding, hair loss and infections. After complete remission and healing, observation is of utmost importance. Neuroblastoma and the treatment of it can sometimes lead to hearing loss, thyroid disorders, growth reduction, learning problems and the risk of falling ill with other cancers later in life.
The picture painted above might seem grim and serious. Any parent receiving the devastating news that their child has a form of cancer needs all our love, support and prayers. Parents should ask their oncology centre for the details of a support group.
