About Neuroblastoma
Neuroblastoma is a type of pediatric cancer that forms in nerve cells called neuroblasts. The exact cause of neuroblastoma is unknown, but there are several factors that may contribute to its development.
Neuroblastoma occurs when a genetic mutation causes developing nerve cells to become cancerous and grow out of control. This may occur either during fetal development or after birth. It usually develops before age 5 and rarely occurs in children over age 10. The reason the genetic mutation happens is unknown.
Non-modifiable risk factors:
- Certain genetic conditions or birth defects, such as Costello syndrome, Noonan syndrome, Neurofibromatosis type 1, Li-Fraumeni syndrome, Beckwith-Wiedemann syndrome, Pheochromocytoma/Paraganglioma syndromes, and ROHHAD syndromes, are associated with an increased risk of neuroblastoma.
- Having a strong family history of neuroblastoma may also be a non-modifiable risk factor. Inherited gene mutations may lead to the development of neuroblastoma at a younger age and may result in multiple tumors in the adrenal glands or nerve tissue.
Modifiable risk factors:
- There are currently no known modifiable risk factors for neuroblastoma that can be changed or controlled.
It's important to note that while these factors may increase the risk of developing neuroblastoma, they do not guarantee its occurrence. If you have concerns about your risk for neuroblastoma, it's best to consult with a healthcare professional who can provide personalized advice based on your individual situation.
Symptoms of neuroblastoma depend on the location of the tumor and the stage of the disease.
- A lump in the abdomen, neck, or chest
- Bulging eyes
- Dark circles around the eyes
- Bone pain
- Swollen stomach
- Trouble breathing in babies
- Painless, bluish lumps under the skin in babies
- Weakness or paralysis (loss of ability to move a body part)
As neuroblastoma progresses or spreads to other parts of the body, other symptoms may occur, including:
- Abdominal distention due to massive liver metastases
- Proptosis and periorbital ecchymosis (bruising around the eyes) from retrobulbar metastasis
- Respiratory compromise in infants due to massive liver metastases
- Pancytopenia (low blood cell counts) from extensive bone marrow metastasis
- Fever, hypertension, and anemia (occasionally found without metastasis)
- Paralysis if the tumor invades through neural foramina and compresses the spinal cord
- Watery diarrhea caused by tumor secretion or protein-losing enteropathy with intestinal lymphangiectasia
It's important to note that these symptoms can also be caused by other conditions, so it's crucial to consult a healthcare professional for an accurate diagnosis.
To diagnose neuroblastoma, healthcare providers commonly perform the following examinations, tests, and procedures:
- Physical exam: The healthcare provider will look for physical signs of a medical problem, such as pain, swelling, or the presence of masses within the body. A neurological exam will check nerve function, reflexes, and coordination.
- Lab tests: Specific lab tests, such as blood draws and urine tests, may be recommended to detect substances that can indicate neuroblastoma.
- Urine catecholamine studies: Tests on a urine sample measures the amounts of certain substances in the urine that can indicate neuroblastoma.
- Blood chemistry studies: A blood sample is checked to measure substances released into the blood by organs and tissues in the body. Blood tests may also include checking blood cell counts and blood chemistry.
- Imaging studies: Imaging tests like X-rays, ultrasounds, CT scans, MRI scans, or MIBG radiotracer scan may be used to visualize the tumor and determine its size and location.
- Genetic tests: These tests analyze samples of blood, saliva, or tissues to identify changes in genes, chromosomes, or proteins that may be associated with neuroblastoma.
- Biopsy: A sample of the tumor tissue may be obtained and examined to confirm neuroblastoma. A bone marrow biopsy may be performed look for signs of neuroblastoma in the bone marrow.
After diagnosing neuroblastoma, additional examinations and tests may be performed to determine the stage or severity of neuroblastoma. Staging is based on how fast the cancer is growing and its spread to other parts of the body.
Other examinations, tests, and procedures may be performed based on individual factors. Diagnosis and staging are important for the healthcare provider to determine a treatment plan.
Treatment depends on the neuroblastoma stage and tumor location. The goals of treatment for neuroblastoma are to remove or destroy the tumor and achieve remission. Remission means there is a decrease or disappearance of cancer signs and symptoms.
In some cases of low-risk neuroblastoma, treatment may not start right away. The child is carefully monitored and treatment started if the tumor grows or symptoms start.
Treatment options for neuroblastoma include:
- Surgery: Surgery may be performed to remove the tumor or as much of the tumor as possible. Chemotherapy sometimes is given to shrink the tumor before surgery, or to destroy remaining cancer cells after surgery. The goal of surgery is to remove the primary tumor while preserving vital organs and maintaining their function. Complete resection of the tumor is important, especially for low-risk neuroblastoma, as it can lead to a high overall survival rate.
- Chemotherapy: Chemotherapy involves using a combination of drugs to kill cancer cells. It is typically used for intermediate-risk or high-risk neuroblastoma cases. Chemotherapy aims to shrink tumors before surgery, destroy remaining cancer cells after surgery, and prevent the spread of cancer to other parts of the body. Chemotherapy is usually given in cycles over several weeks or months.
- Targeted therapy: Targeted therapy involves using drugs or substances that specifically attack cancer cells with minimal harm to normal cells. In the case of neuroblastoma, targeted therapy may include anti-GD2 immunotherapy or iodine 131-MIBG therapy, which deliver treatments directly to neuroblastoma cells.
- Radiation therapy: Radiation therapy utilizes high-energy rays, such as X-rays, to target and kill cancer cells or stop them from growing or multiplying. It may be used to help prevent cancer from returning after other treatments.
- Stem cell transplant: Stem cell transplant may be used after using high-dose chemotherapy to destroy cancer cells. High-dose chemotherapy may damage bone marrow stem cells. Stem cell transplant replaces damaged bone marrow stem cells with healthy ones that can mature into blood cells needed to support the immune system.
It is important to note that the healthcare professional recommends specific medications, therapies, and therapeutic procedures based on individual patient factors. Medication dosing may be affected by many factors, so it's crucial to consult with a healthcare professional for personalized advice. Side effects can occur, so it's essential to discuss potential risks with a healthcare professional.