Topic: Neuroblastoma

Neuroblastoma is a malignant tumor that starts in certain types of very primitive nerve cells found in an embryo or fetus. This type of cancer occurs in infants and young children. It is rarely found in children older than 10 years. Neuroblastomas are cancers that start in early nerve cells of the sympathetic nervous system (sympathetic neuroblasts), so they can be found anywhere along this system. The sympathetic nervous system controls body functions, such as heart rate and blood pressure, digestion, and levels of certain hormones. According to www.americancancersociety.org 1 out of 3 neuroblastomas start in the adrenal glands, 1 out of 3 begins in sympathetic nerve ganglia in the abdomen, and most of the rest start in sympathetic ganglia near the spine in the chest or neck or in the pelvis. There is a wide spectrum of how neuroblastomas can behave. Sometimes, in very young children, the cancer cells die without any cause and the tumor goes away on its own. In other cases, the cells sometimes mature on their own into normal ganglion cells and stop dividing.
Normal body cells grow, divide, and die in an orderly fashion. During the early years of a person's life, normal cells divide faster to allow the person to grow. After the person becomes an adult, most cells divide only to replace worn-out or dying cells or to repair injuries. Cancer begins when cells in a part of the body start to grow out of control. Cancer cells can invade other tissues, something that normal cells cannot do. Growing out of control and invading other tissues are what makes a cell a cancer cell. Cells become cancer cells because of damage to DNA. DNA is in every cell and directs all its actions. In a normal cell, when DNA gets damaged the cell either repairs the damage or the cell dies. In cancer cells, the damaged DNA is not repaired, but the cell doesn’t die like it should. Instead, this cell goes on making new cells that the body does not need. These new cells will all have the same damaged DNA as the first cell does. People can inherit damaged DNA, but most DNA damage is caused by mistakes that happen while the normal cell is reproducing or by something in our environment. Sometimes the cause of the DNA damage is something obvious, like cigarette smoking. But often no clear cause is found. Cancer cells often travel to other parts of the body, where they begin to grow and form new tumors that replace normal tissue. This process is called metastasis. It happens when the cancer cells get into the bloodstream or lymph vessels of our body.

Neuroblastomas are usually found as a result of signs or symptoms that a child is having. One of the most common signs of a neuroblastoma is an unusual lump or mass, general malaise. The mass is usually found in the child's abdomen, causing it to swell. The child may not want to eat (which can lead to weight loss). Sometimes, swelling from a tumor in the abdomen may affect parts of the body that do not contain cancer, especially the legs and, in males, the scrotum. Swelling may be seen in the upper chest, headaches, dizziness, and a change in consciousness if it affects the brain. The tumor may also press on the throat or windpipe, which may make it hard for the child to breathe or swallow.Lymphadenopathy in the cervical and supraclavicular area may be seen also. Neuroblastomas that press on certain nerves in the chest or neck can sometimes cause other symptoms, such as a drooping eyelid and small pupil. Inability to feel or move the arms and legs may also be affected. Statistics state (www.americancancersociety.org) 2 out of 3 cases of neuroblastoma have spread to other parts of the body by the time they are found. Neuroblastoma frequently spreads to bones, being referred to as a “silent” tumor in “Wong’s Essentials of Pediatric Nursing 8th edition” (clinical companion). A child who can talk may complain of pain in the bones. The pain may be so bad that the child limps or refuses to walk. If it spreads to the backbone, tumors may press on the spinal cord and cause weakness, numbness, or paralysis in the arms or legs. Spread to the bones around the eyes can lead to bruising around the eyes or cause an eyeball to stick out slightly. Blue or purple bumps that look like small blueberries may be a sign of spread to the skin.
If the cancer spreads to the bone marrow (the inner part of certain bones that makes blood cells), the child may not have enough red blood cells, white blood cells, or blood platelets. These shortages of blood cells can result in tiredness, irritability, weakness, frequent infections, and excess bleeding from small cuts or scrapes. A special widespread form of neuroblastoma (known as stage 4S) occurs only during the first few months of life. In this special form, the neuroblastoma has spread to the liver, to the skin, and/or to the bone marrow (in small amounts). The liver can become very large. Despite the fact that the cancer is already widespread when it is found, stage 4S neuroblastoma is very treatable (often shrinking or going away on its own), and almost all children with this form of neuroblastoma can be cured. Neuroblastoma is one of the few cancers in children that release hormones that can cause problems with distant tissues and organs, even though the cancer has not spread to those tissues or organs. These problems are called paraneoplastic syndromes.
Symptoms of paraneoplastic syndromes can include:
• Constant diarrhea
• Fever
• High blood pressure (causing irritability)
• Rapid heartbeat
• Reddening (flushing) of the skin
• Sweating
To assist with diagnosis the physician with take a complete medical history and perform a physical. If the results are suggestive of neuroblastoma other tests such as; blood and urine tests, imaging tests (Xray,Nuc-Med, CT,PET US,MIBG), and biopsies may be performed. Neuroblastoma can often be found by detecting catecholamines in the blood or urine. Sympathetic nerve cells normally release hormones called catecholamines, such as epinephrine (adrenaline) and norepinephrine. Eventually the body breaks these down into metabolites (smaller pieces), which are then passed out of the body in the urine.

In most cases, neuroblastoma cells make enough catecholamines to be detected by blood or 24 hour urine tests. The 2 catecholamine metabolites most often measured are: Homovanillic acid (HVA), Vanillylmandelic acid (VMA). Additional studies that may be ordered are; blood tests to check blood cell counts, liver and kidney function, and the balance of salts (electrolytes) in the body. A urinalysis (urine test) may also be done to further check kidney function.
Treatment Options:
The approach to treatment of neuroblastoma depends on the stage of the cancer, the child's age, and other factors such as the prognostic markers mentioned previously. The types of treatment used may include:
• Surgery (diagnosis and treatment)
• Chemotherapy (high dose) followed by autologous stem cell transplantation (being studied for children with very high risk tumors)
• Retinoid therapy
• Radiation therapy
• High-dose chemotherapy/radiation therapy and stem cell transplant
• Immunotherapy
In many cases, more than one type of treatment is needed. Surgery can be used both to help diagnose neuroblastoma and to treat it. For smaller tumors that have not spread, surgery is often the only treatment that is needed. After neuroblastoma is diagnosed, surgery is often used to try to remove as much of the tumor as possible. Even if the tumor cannot be completely taken out, treatment with chemotherapy (and sometimes radiation therapy) after removing most of the cancer may result in a cure. Sometimes surgery is repeated after other treatments (chemotherapy and/or radiation therapy) to check the results of therapy and to remove any remaining cancer if possible. If the tumor is very big, chemotherapy may be used before surgery. This can shrink the tumor and make it easier to remove completely.

Interventions:
Infection prevention, emotional support for child and family, cancer support groups, preparation for child and family related to procedures, administer therapy, manage side effects, prevent complications (monitor pt, labs, VS, I/O, assessment), educate the child and family on medications, side effects to expect, safety related to neutropenia/activity, monitor nutritional status and weight, manage central live/IV , encourage fluids, provide education/handouts on chemotherapy/radiation if included in therapy to help with anticipation of side effects. Offer age appropriate interventions.