Neuroblastoma

Neuroblastoma arises from neuroblasts, primitive cells of the sympathetic nervous system that normally mature into nerve tissue during development. It is mainly a cancer of infants and young children, and most children are diagnosed before the age of five. The tumour most commonly starts in the adrenal gland, which sits on top of the kidney, but it can also begin in nerve tissue in the neck, chest, abdomen or pelvis.

Neuroblastoma behaves very differently from one child to another. Some tumours in very young infants can shrink on their own or with minimal treatment, while others are more aggressive and have already spread at diagnosis. Because of this variability, treatment is tailored to each child after careful assessment by a specialist children's cancer service.

Neuroblastoma often presents as a painless lump or swelling in the abdomen, sometimes noticed during bathing or dressing. Other signs depend on where the tumour is and can include tiredness, poor appetite, bone pain, a limp, or bruising around the eyes. Some tumours are found incidentally on scans done for another reason.

Diagnosis usually involves:

• Ultrasound, CT or MRI to define the tumour and its relationship to nearby blood vessels and organs
• Urine tests for catecholamines, chemicals released by many neuroblastomas
• A biopsy so that the tumour can be examined under the microscope and its genetic features tested
• Bone marrow tests and specialised nuclear medicine scans (such as an MIBG scan) to check whether the disease has spread

These results are brought together to assign a risk group, which guides the treatment plan.

Neuroblastoma is treated within a multidisciplinary children's cancer service, where paediatric oncologists, surgeons, radiation oncologists, radiologists, pathologists and nursing and allied health staff plan care together. Your child's treatment depends on their risk group.

For low-risk tumours, surgery alone may be all that is required, and in some infants careful observation is appropriate. For intermediate and high-risk disease, chemotherapy is usually given first to shrink the tumour and treat any spread, with surgery planned once the tumour is smaller and safer to remove. High-risk neuroblastoma is treated with intensive combinations that may include chemotherapy, surgery, high-dose therapy with stem cell rescue, radiotherapy and immunotherapy.

The surgeon's role is coordinated with the oncologists so that the timing of the operation fits within the overall treatment plan.

The aim of surgery is to remove as much of the tumour as can be done safely. Neuroblastomas often wrap around major blood vessels supplying the kidneys, bowel and liver, so the operation is planned in detail using your child's scans. Your child's surgeon will explain the approach, which is usually an open operation through the abdomen, chest or neck depending on where the tumour lies. Keyhole techniques may be suitable for some small tumours.

The surgery is performed under general anaesthesia with a paediatric anaesthetist. Lymph nodes near the tumour are often sampled or removed for staging. In some children a small amount of tumour is deliberately left where removing it would put a vital structure at risk, and this is treated with the other therapies in the plan.

After the operation your child will be cared for in hospital, sometimes initially in a paediatric intensive care or high-dependency unit, until they are eating, comfortable and moving around. The length of stay depends on the size and location of the tumour, and the treating team will guide you on wound care, pain relief and activity at home.

Pathology results from the operation are reviewed at the multidisciplinary meeting and help decide whether further chemotherapy, radiotherapy or other treatment is needed. Follow-up includes regular clinical reviews, urine tests and scans over several years to check for any recurrence and to monitor growth and development. Long-term follow-up clinics also look after children who have received intensive treatment, watching for late effects and supporting the family throughout.