Overview

Neuroblastoma is a rare type of cancer that mostly affects babies and young children.

It develops from specialised nerve cells (neuroblasts) left behind from a baby's development in the womb.

Neuroblastoma most commonly occurs in one of the adrenal glands situated above the kidneys, or in the nerve tissue that runs alongside the spinal cord in the neck, chest, tummy or pelvis.

It can spread to other organs such as the bone marrow, bone, lymph nodes, liver and skin.

Title
neuroblastoma

Symptoms

The symptoms of neuroblastoma vary depending on where the cancer is and whether it has spread.

The early symptoms can be vague and hard to spot, and can easily be mistaken for those of more common childhood conditions.

Symptoms can include:

  • a swollen painful tummy, sometimes in association with constipation and difficulty passing urine
  • breathlessness and difficulty swallowing
  • a lump in the neck
  • blueish lumps in the skin and bruising, particularly around the eyes
  • weakness in the legs and an unsteady walk, with numbness in the lower body, constipation and difficulty passing urine
  • fatigue, loss of energy, pale skin, loss of appetite and weight loss
  • bone pain, a limp and general irritability
  • rarely, jerky eye and muscle movements
Causes

The cause is unknown. There are very rare cases where children in the same family are affected, but generally neuroblastoma doesn't run in families.

Diagnosis

A number of tests may be carried out if it's thought your child could have neuroblastoma.

These tests may include:

  • a urine test – to check for certain chemicals produced by neuroblastoma cells that are found in urine
  • scans – such as ultrasound scans, CT scans and MRI scans of various parts of the body to look at these areas in detail
  • an mIBG scan – this involves the injection of a substance taken up by neuroblastoma cells
  • a biopsy – the removal of a sample of cells from the tumour tissue for examination under a microscope so the type of cancer can be identified; the sample is usually removed under general anaesthetic using a special needle
  • bone marrow biopsies – to see if there are cancer cells in the bone marrow

Once these tests have been completed, it will usually be possible to confirm if the diagnosis is neuroblastoma and determine what stage it is.

As with most cancers, neuroblastoma is given a stage. This indicates if it has spread and, if so, how far.

The staging system used for neuroblastoma is:

·       stage L1 – the cancer is just in one place and hasn't spread, and can be removed by surgery

·       stage L2 – the cancer is in one place and hasn't spread, but can't be removed safely by surgery

·       stage M – the cancer has spread to other parts of the body

·       stage Ms – the cancer has spread to the skin, liver or bone marrow in children aged less than 18 months

Knowing the stage of your child's neuroblastoma will allow doctors to decide which treatment is best.

Treatment

The main treatments for neuroblastoma are:

  • surgery to remove the cancer – sometimes this may be all that's needed
  • chemotherapy (where medication is used to kill cancer cells) – this may be the only treatment needed or it may be given to shrink the cancer before surgery
  • radiotherapy (where radiation is used to kill cancer cells) – this may sometimes be used after surgery to destroy any remaining cancer cells in the affected area
  • high-dose chemotherapy followed by a stem cell transplant – where stem cells from your child are collected, frozen and stored prior to intensive chemotherapy, and are given back to them afterwards
  • immunotherapy – where a medication that directly targets the neuroblastoma cells is given, although this isn't used routinely yet

Some babies and infants less than 18 months old with either stage L1 or Ms neuroblastoma who have no symptoms may not need any treatment, as the cancer can sometimes go away on its own.

The outlook for neuroblastoma varies considerably, and is generally better for younger children whose cancer hasn't spread. Your doctors will be able to give you more specific information about your child.

Almost half of neuroblastomas are a type that can return despite intensive treatment. Further treatment will often be necessary in these cases.