Neuroblastoma is a rare childhood cancer usually occurs in very young children under the age of 5. It is the most common solid tumour in infants. It starts from a type of cells called neuroblasts which are present in the neural crests. The tumour can occur from any part of the neural crests in the body but more commonly occurs in the abdomen, particularly in the adrenal gland.Risk factors for developing a neuroblastoma include a family history of the condition, genetic disorders such as Neurofibromatosis Type 1, Beckwith-Wiedemann syndrome, Li-Fraumeni syndrome and Costello symdrome.
Symptoms of a neuroblastoma include presence of an abdominal mass associated with discomfort or pain. If it has spread to other parts of the body, it can cause fever, weight loss, failure to thrive and pain if there is spread of cancer into the bones.
Investigations to diagnose the cancer include a scan like an MRI or CT scan to look at the primary tumour and to look for spread of cancer to other areas in the body. Other tests include a bone scan to look for spread into the bones, Iodine 123-MIBG scan and a bone marrow biopsy. Genetic tests to look for MYCN genes helps in determining whether the tumour will grow fast or slow.
Staging of a neuroblastoma is as follows.
|Stage 1 and 2||The cancer is confined to one area without any spread|
|Stage 3||The cancer has spread to nearby structures but not far away|
|Stage 4||The cancer has spread to distant parts of the body|
|Stage 4S||This stage is limited to infants below 1 year, have primary tumour that is removable with surgery and have metastases that are confined to the liver, skin and bone marrow.|
Treatment of a neuroblastoma is dependent on the stage and risk of cancer at diagnosis. Also, the age, fitness and the risk group of the disease play a part in determining a treatment strategy. Treatment options include surgery, chemotherapy and radiotherapy. For patients with disease confined to only one area and are considered low risk surgery may be the only treatment needed. In other low risk patients, surgery and chemotherapy are used. In patients with disease that is too large to operate, chemotherapy first followed by surgery can be done. In patients with stage 4S disease, observation alone may be an option. If chemotherapy is used, the drugs selected can include Cisplatin or Carboplatin, Doxorubicin, Etoposide and Cyclofosfamide.
For children with intermediate risk neuroblastoma, a combination of chemotherapy and surgery are usually done. This can be surgery followed by chemotherapy or vice versa.
For children with high risk neuroblastoma, options of treatment include chemotherapy, surgery, radiotherapy as well as high dose chemotherapy followed by stem cell transplant. Treatment is more aggressive for high risk patients and can be divided into induction, consolidation and maintenance phases. All the above mentioned options may be used for the same patient. Also, biological therapy with Dinutuximab can be used.