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Neuroblastoma is a common childhood cancer that originates from a part of the nervous system called the autonomic nervous system. This system regulates a person's heart rate, blood pressure and digestion. It usually arises in the nerves of the chest or abdomen and is most common in the adrenal glands located above the kidneys. Neuroblastoma is the third most common childhood cancer and is the most common solid tumor in children that is found outside of the skull.
Neuroblastoma usually affects children who are less than 8 years old. The average age of diagnosis is 2 years old. Neuroblastoma accounts for 7.8% of all childhood malignancies. Of all children diagnosed with neuroblastoma, 40% are found in children younger than 1 year, 35% are in children aged 1-2 years, and 25% are in children older than 2 years.
The size and the extent of the neuroblastoma will determine which signs and symptoms a child will have. If the neuroblastoma is located in the abdomen, the first signs and symptoms may be a large belly, a sensation of fullness, vomiting, abdominal pain, weight loss, poor appetite, diarrhea, changes in urinary habits, or leg swelling. If the neuroblastoma is in the chest, the child may have wheezing, respiratory distress, swelling in the face, or dropping eyelids. Other general symptoms may include fever, irritability, bone pain, and rarely hypertension (high blood pressure). If the neuroblastoma has spread, different symptoms may be present. Below is a list of symptoms that might be present if it has spread to specific parts of the body.
Bone: the child may limp and have bone pain.
Anemia: a result of decreased number of red blood cells.
Bruising: a result of decreased number of platelets.
Infections: a result of decreased number of white blood cells.
Eyes: the child may appear to have "raccoon eyes" or dark circles surrounding the eyes. Another symptom is opsoclonus, which are irregular, jerky, movements of the eyes in vertical and horizontal directions.
Gastrointestinal tract: bleeding, diarrhea, nausea, constipation and obstruction.
Myoclonus: twitching or spasm of muscles.
Liver: enlarged liver.
Spinal cord: weakness in the arms or legs.
Skin: a bluish rash commonly described as "blueberry-muffin" may be present.
Ninety percent of neuroblastomas can produce hormones like epinephrine, which can increase the heart rate and cause anxiety.
The cause for the genetic mutation that leads to neuroblastoma is unclear. Due to the early age with which children acquire the neuroblastoma, researchers believe that the mutation occurs during pregnancy or even during conception. An association has been noted with other genetic syndromes including Beckwith-Weideman, Fetal Alcohol Syndrome, Pheochromocytoma, Neurofibromatosis Type 1 and Hirschsprungs Disease.
The diagnosis is usually made by initial imaging studies such as a CT (computed tomography) or MRI (magnetic resonance imaging) scan. Since epinephrine may be over-produced, a urine sample can be obtained to look for breakdown products of epinephrine. A biopsy of the mass is usually done to confirm the diagnosis. The tissue is then examined under a microscope and the tissue is sent for other confirmatory tests. There are a variety of other special tests that can be done to help confirm the diagnosis of neuroblastoma. Such tests are the MIBG scan, chromosomal studies with markers like CD44 and GD2, and a bone scan to rule out a metastatic (generalized) spread of tumor. The blood work-up usually begins with a complete blood count (CBC) to determine the severity of the anemia and risk of bleeding. A bone marrow biopsy should be performed to see if the neuroblastoma has spread to the bone marrow. Once confirmed, a piece of the neuroblastoma should be analyzed for amplification of a particular gene called n-myc.
Surgical removal is the best chance for a cure. Chemotherapy usually is needed for children who are older than 1 year of age and for children with stage II and greater. Some chemotherapy medicines used to treat neuroblastoma are: daunorubicin, cyclophosphamide, carboplatin, and epotoside. Radiation therapy is needed for advanced stages of the disease (III and up) and if the child is older than 1 year. Another form of treatment is an autologous bone marrow transplant. The child's own bone marrow or mobilized blood cells is collected, possibly treated with chemotherapy drugs and then given back to the child to reconstitute the blood making elements in the body. An auto bone marrow transplant has fewer risks of immune system incompatibility than a transplant from a sibling or family member. Some newer treatment options include immunotherapy with monoclonal antibodies, IL-2 vaccines, and vitamin A (retinoic acid).
Treatment depends on the stage of the tumor. There are 6 stages of neuroblastoma:
STAGE I- localized tumor that can be completely removed surgically. The lymph nodes are tested to see if the cancer has spread (metastasis) and in this stage all lymph nodes are negative, which means that the cancer has not spread.
STAGE IIA- localized tumor that can only be partially removed surgically. The lymph nodes on the same side of the tumor are negative.
STAGE IIB- localized tumor with or without complete surgical removal. The lymph nodes on the same side are positive (the cancer has spread) but the lymph nodes of the opposite side (contralateral) are negative, which means that the cancer has not spread to the opposite side of the body.
STAGE III- inoperable tumor that has crossed to the other side of the body, or a localized tumor with positive lymph nodes on the opposite side, or a tumor that is in the middle of the body and has spread to both sides which makes it inoperable.
STAGE IV- primary tumor that is considered advanced and has spread (metastasized) to distant lymph nodes, bone, bone marrow, liver, skin, and/or other body organs.
STAGE IV S- localized tumor that has traveled to the skin, liver, and/or bone marrow in infants less that 1 year old.
The prognosis is different for every stage of the disease. The overall 5-year survival rate for children with neuroblastoma is approximately 55%. Infants younger than 1 year of age have a better survival rate than do children older than 1 year. Five-year survival rate for children younger than 1 year is 83%, children 1 to 4 years is 55%, and for children 5 to 9 years is 40%. Pediatric cancer centers continuously work on improving treatment options to optimize survival.
This is a good website with a thorough review of Neuroblastoma, explanations of the various stages, and treatment options as well as links to support networks as well as ongoing clinical trials.
Cancer Help Online
Links include informational sites, personal stories, organizations dedicated to Neuroblastoma, and ongoing research.
Dambro, Mark M.D. GRIFFITH'S 5-MINUTE CLINICAL CONSULT- 11th edition (2003)
Rudolph et.al RUDOLPH'S PEDIATRICS- 21st edition (2002)
The Harriet Lane Handbook: A Manual for Pediatric House Officers, 16th edition
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