Neuroblastoma: Symptoms, causes, diagnosis, treatments
Generally, cancer begins with an inherited mutation that allows cells to continue growing without responding to stop signals like normal cells.
Neuroblastoma: Symptoms, causes, diagnosis, treatments
Neuroblastoma is cancer that develops from nerve cells found in certain areas of the body. Neuroblastoma most commonly arises in and around the adrenal glands, having a similar origin to neurons. However, neuroblastoma can also develop in other areas of the abdomen, neck, chest, and pelvis.
Neuroblastoma most commonly affects children 5 years of age or younger, although it can rarely occur in older children.
Some forms of neuroblastoma improve on their own, while others may require multiple treatments. The choice of treatment for neuroblastoma depends on many factors
The signs and symptoms of neuroblastoma vary depending on which part of the body is affected.
Neuroblastoma in the abdomen
The most common form can cause signs and symptoms such as:
The subcutaneous mass is not tender to the touch.
Change in bowel habits, such as diarrhea.
Swelling in the legs.
Neuroblastoma in the chest
May cause signs and symptoms such as:
Eye changes, including drooping eyelids and irregular pupil size.
Signs and symptoms that may indicate neuroblastoma include
Tumors of subcutaneous tissue.
The eyeball appears to protrude (proptosis).
Dark circles, similar to bruises around the eyes.
Unexplained weight loss.
Contact your doctor if you have any worrying signs or symptoms. Refers to any change in behavior or habits.
Generally, cancer begins with an inherited mutation that allows cells to continue growing without responding to stop signals like normal cells. Cancer cells grow and multiply out of control. Abnormal cells accumulate to form a mass (tumor). Cancer cells can invade nearby tissue and can break away from the original tumor and spread to other parts of the body (metastasis).
Neuroblastoma begins in neuroblasts (neuroblasts) - nerve cells that are immature in the fetus. As the fetus matures, neuroblasts eventually turn into the neurons, fibers, and cells that make up the adrenal glands. The majority of neuroblasts are mature at birth, although research has found a small number of neuroblasts are immature in infants. In most cases, these neuroblasts will mature or disappear. Others, however, form a tumor - neuroblastoma.
It is not clear what causes the genetic mutation that initially leads to neuroblastoma. However, because neuroblastoma often affects young children, researchers believe the mutation occurs during pregnancy or shortly after birth.
The danger elements
The only known factor that increases the risk of neuroblastoma is a family history of cancer. However, familial neuroblastoma is thought to represent a very small number of neuroblastoma cases. In most cases of neuroblastoma, the cause is unknown.
Complications of neuroblastoma can include:
Metastasis. Neuroblastoma can metastasize to other parts of the body, such as the lymph nodes, bone marrow, liver, skin, and bones.
Compression of the spine. The tumor can grow and press on the spinal cord, causing compression of the spine. Compression of the spine can cause pain and paralysis.
Signs and symptoms caused by secretory tumors. Neuroblastoma can secrete a chemical that irritates normal tissues, causing signs and symptoms known as paraneoplastic syndromes. Paraneoplastic rarely occurs in people with neuroblastoma, causing rapid eye movements and difficulty with coordination. The rare syndrome is caused by abdominal swelling and diarrhea.
Tests and diagnostics
Tests and procedures used to diagnose neuroblastoma include:
Clinical examination. The doctor proceeds to check for any signs and symptoms. The doctor will ask questions about the patient's habits and behavior.
Check urine and blood. May indicate the cause of any signs and symptoms you are experiencing. Urine tests may be used to check for certain chemicals that are overproduced by neuroblastoma cells.
Check pictures. Imaging tests can reveal masses that may indicate a tumor. Imaging tests may include X-rays, ultrasound, computed tomography (CT scan), and magnetic resonance imaging (MRI).
Remove tissue for testing. If a tumor is found, the doctor may remove a sample of tissue for testing (biopsy). Specialized examination of the tissue sample can reveal the types of cells involved in the tumor and the specific genetic characteristics of the cancer cells. This information helps your doctor come up with a treatment plan.
Remove a sample of bone marrow for testing. You may also undergo a bone marrow biopsy to see if the neuroblastoma has spread to the bone marrow. To remove the test bone marrow, the surgeon inserts a needle into the pelvis and drains the marrow.
Once neuroblastoma is diagnosed, your doctor may order further testing to determine how far cancer has spread to distant organs. Imaging tests used for staging include X-rays, computed tomography (CT) scans, or MRIs.
Using information from the procedure, doctors stage neuroblastoma. The stages of neuroblastoma include:
Stage I. Neuroblastoma at this stage is in situ, which means it is limited to one area and can be completely removed with surgery. Lymph nodes connected to the tumor may show signs of cancer, but other lymph nodes do not have cancer.
Phase IIA. Neuroblastoma at this stage is localized, but may not be easily removed by surgery.
Stage IIB. Neuroblastoma at this stage is localized and may or may not be easily removed surgically. Both lymph nodes connect to the tumor and nearby lymph nodes contain cancer cells.
Phase III. Neuroblastoma at this stage is considered advanced, and it is not possible to completely remove the tumor through surgery. Tumors may be larger at this stage. Lymph nodes may or may not contain cancer cells.
Stage IV. Neuroblastoma at this stage is considered advanced and has spread (metastasized) to other parts of the body.
IVS stage. This stage is a special type of neuroblastoma that doesn't behave like other forms of neuroblastoma, although it's not clear why. Neuroblastoma stage IVS only applies to children older than 1 year. IVS indicates that the neuroblastoma has spread to another part of the body - most often the bone marrow, skin, or liver. Despite the extent of neuroblastoma, infants with this stage have a good chance of recovery. Neuroblastoma at this stage sometimes improves on its own and usually does not require treatment.
Treatments and drugs
Doctors choose a treatment plan based on several factors that affect prognosis. Factors include age, stage of cancer, the type of cells involved in cancer, and any abnormalities in chromosomes and genes. Using this information, the doctor classifies cancer as low risk, intermediate-risk, or high risk. Treatment or combination of treatments for neuroblastoma depends on the type of risk.
Surgeons use scalpels and other surgical tools to remove cancer cells. In children with low-risk neuroblastoma, surgery to remove the tumor may be the only treatment needed. Whether the tumor can be completely removed depends on its location and its size. Tumors that are attached to nearby vital organs - such as the lungs or spinal cord - can be too dangerous to remove. Neuroblastoma is medium- and high-risk, and the surgeon may try to remove as much of the tumor as possible. Other treatments, such as chemotherapy and radiation therapy, may then be used to kill any remaining cancer cells.
Chemotherapy uses chemicals to kill cancer cells. Chemotherapy rapidly targets cells in the body, including cancer cells. Unfortunately, chemotherapy also damages healthy cells quickly, such as those in hair follicles and in the digestive system, which can cause side effects.
Children with low-risk neuroblastoma that cannot be operated on may undergo chemotherapy. Sometimes chemotherapy is administered before surgery to shrink the tumor. In other cases, chemotherapy may be the only treatment.
Children with moderate-risk neuroblastoma often receive a combination of chemotherapy drugs before surgery to improve the chance that the entire tumor can be removed.
Children with high-risk neuroblastoma often receive high-dose chemotherapy drugs to shrink the tumor and kill any cancer cells that have spread elsewhere in the body. Chemotherapy is often used before surgery and before a bone marrow stem cell transplant.
Radiation therapy uses high doses of energetic particles to kill cancer cells. Radiation therapy mainly affects the area where it is targeted. Radiation treatment tries to protect healthy cells near cancer, but some healthy cells can be damaged by radiation. Effects depend on where the radiation is directed and how much radiation.
Children with low- or intermediate-risk neuroblastoma may receive radiation therapy if surgery and chemotherapy have not been helpful. Children with high-risk neuroblastoma may receive radiation therapy after chemotherapy and surgery, to prevent cancer from coming back.
Stem cell transplant
Children with high-risk neuroblastoma may receive a transplant using their own blood stem cells (autologous stem cell transplant). Bone marrow produces adult stem cells and develops into red blood cells, white blood cells, and platelets. Undergo filtration and collect stem cells from the blood. Then, high-dose chemotherapy is used to kill the remaining cancer cells in the body. The stem cells are then injected into the body, where new healthy blood cells can be formed.
Long-term side effects and after cancer treatment
Children are more likely to survive cancer and live to adulthood, and doctors are becoming increasingly aware of the long-term side effects of cancer treatment. Doctors advise childhood cancer survivors to have regular follow-up visits, understanding the long-term and possible side effects after cancer treatment.
Side effects vary depending on treatment but can include growth failure, thyroid problems, second cancer, and infertility, which can be caused by chemotherapy drugs. Difficulty thinking and dealing with problems can be long-term side effects of radiation, especially brain or body radiation.
Coping and supporting
When diagnosed with cancer, it is common to feel a range of emotions from shock and disbelief to guilt and anger. In this emotional context, it is expected to make decisions about treatment. And while trying to sort it all out, friends and family may be called upon to stay updated and find out if they can help. If you're feeling lost, you can try:
Collect all possible information. Learn all you can about neuroblastoma. Talk to the health care team. Keep a list of questions to ask at follow-up appointments.
Organize a support network. In the coming weeks and months, undergoing tests and procedures will be time-consuming and may require travel to a specialist medical center. Relatives can accompany you to see a doctor or sit next to you in the hospital.
Take advantage of resources for children with cancer. Find special resources for families of children with cancer. Social workers and other members of the health care team can tell you about available plans. Support groups for parents and siblings get in touch with people who understand what you're feeling.
Stay as normal as possible. Young children cannot understand what is happening while undergoing cancer treatment. To help cope, try to maintain a normal routine as much as possible. Try to schedule a nap time each day. Have regular meals. Allow time to play when you feel like it. Ask your health care team about other ways to comfort.