What is Neuroblastoma?
Neuroblastomais a type of tumor that arises from cells of the sympathetic nervous system and adrenal gland.
Neuroblastoma is seen mostly in children. It is the third most common childhood cancer, after leukemia and brain tumors.Since neuroblastomas arise from cells of the nervous system, it can occur in any area with sympathetic nerve tissues, including the adrenal gland (46%), the chest next to the spine (15%), the pelvis (4%), and the head and neck (3%).
What are the symptoms of Neuroblastoma?
The symptoms of neuroblastoma vary according to the location of the main tumor and whether or not it has spread to other places in the body.
When the tumor arises inside the belly, there may be a mass or firmness felt during a physical exam.When they occur in the chest, it may compress the windpipe (or trachea, which is a major airway in the neck and upper chest) and create coughing or difficulty breathing.If it is near the spinal cord, it can create a loss of sensation or motor function below the level of involvement. Spread to the lymph nodes can create enlarged lumps or bumps on the body, while spread to the bone marrow can create fatigue and an inability to fight infection.
Some common symptoms that children can experience with neuroblastomas regardless of its location include weight loss, fever, abdominal disturbances, diarrhea, high blood pressure, irritability, pain of bone and joints, involuntary movements, or inability to stand up or to walk.
What are the tests to be performed?
Neuroblastoma will require extensive work up.
- Blood and urine investigations; Apart from routine tests, blood and urine may be analyzed to detect the hormones produced by neuroblastoma. They are helpful in monitoring the disease as well as picking up a recurrence.
- Imaging studies; Sonography, X-rays, CT scan of the involved area are usually required. Sometimes, MRI is also required when there is neurological involvement.
- MIBG scan; Iodine-123-labeled metaiodobenzylguanidine (MIBG), a radiolabeled marker that is processed by the adrenal gland, is a nuclear medicine test that is useful in identifying the tumor and metastases.
- Bone marrow examination is required to confirm the presence or absence of neuroblastoma in it.
- Biopsy of the tumor (operative or tru-cut) and histopathological examination with immunohistochemistry and molecular marker analysis are required for diagnosis and prognosis.
What is the treatment?
Surgery, chemotherapy, radiotherapy and stem cell therapy are the various modalities of treatment required in combination of one another depending on the age of the child, location and extent of the disease, presence or absence of the metastasis, aggressiveness and biological behavior of the tumor.
Surgery is an essential component of the treatment as tumor removal (total or maximum possible) is necessary for favorable results.
What are the risks of surgery?
Bleeding, infection, residual tumor and intestinal obstruction are the possible risks.
What is the long term prognosis?
Overall, Neuroblastomas that are 1) diagnosed under the age of 18 months, 2) located in the chest, neck or lower spine and 3) do not have lymph node involvement all have more favorable outcomes. In these low-risk groups, there is a 90% long-term survival rate.
In cases where the tumor is 1) too large, 2) there is lymph node involvement or 3) distant metastasis (spread of cancer to other organs), outcomes may not be as good and chemotherapy and/or radiotherapy may be the only option for treatment.
A long term follow up is essential for children with neuroblastoma.