Ganglioneuroblastoma

Related Terms

Benign tumor, biopsy, bone marrow, cancer, childhood cancer, childhood neoplasm, extracranial, ganglia, ganglion, ganglioneuroblastoma, ganglioneuroblastoma intermixed, ganglioneuroblastoma nodular, ganglioneuroma, intermediate tumor, malignant tumor, metastatic, needle aspiration, nerve tissue, nervous system, neuroblastoma, neuroblasts, pediatric cancer, solid tumor, tumor.

Background

Ganglioneuroblastoma is a tumor or abnormal tissue growth that is generated from nerve tissue.
The name of this disease gives clues to where it occurs and what it affects. Ganglia are masses of nerve cells, "neuro" means nerve, and the term "blastoma" means a cancer that affects immature or developing cells.
The nervous system includes the brain, spinal cord, and all of the nerves that communicate with the rest of the body. The nervous system is used for thinking, feeling, moving, and other functions. Because this type of cancer forms on the clusters of nerve cells, it may be found in almost any part of the body.
Sometimes the tumor grows slowly, while other times, it grows and spreads quickly. It is classified as an intermediate tumor for this reason. It is more severe than a benign tumor, which is typically less likely to spread and is slow to grow, but less aggressive than a malignant tumor, which typically has rapid growth, is more likely to spread, and advances quickly. Therefore, a ganglioneuroblastoma can be localized or it may have metastasized (migrated from the area of origin to other parts of the brain or body).
The cells of a ganglioneuroblastoma have a dual feature. Some are immature and poorly differentiated, less distinct in form and function, and look dissimilar to the tissue from which they originated. Others are mature ganglion cells, specialized with distinct form and function, and look similar to the tissue from which they originated. Cell differentiation can indicate the likelihood of a tumor to remain localized or metastasize. Well-differentiated cells, those that look similar to the tissue from which they originated, are less likely to metastasize.
Some places where ganglioneuroblastomas may metastasize include bones, bone marrow, lymph nodes, the abdomen, the liver, the chest, and the serum (blood).
The cause of ganglioneuroblastomas is unknown, but age and genetics are thought to play a role.
Ganglioneuroblastoma is a rare tumor, with a majority of cases seen in young children up to four years of age. It has an incidence rate of less than five cases per 1,000,000 children. This incidence rate is highest during the first year of life, and it is not more prevalent in one race than another.

Signs and symptoms

The signs and symptoms for ganglioneuroblastoma may be different for each child, depending on where the cancer is, how big it is, and where it has spread. Ganglioneuroblastomas may also spread to other parts of the body, causing other symptoms. Symptoms of ganglioneuroblastomas include pain, proptosis (eye protrusion), abdominal distention, ecchymosis (purple discoloration under the skin caused by a ruptured blood vessel), malaise (a general feeling of bodily discomfort), weight loss, anorexia, irritability, and fever.
Pain is the most common symptom in patients, stemming from bone, bone marrow, or abdominal involvement.
A hard lump is the most common sign. The lump is often found in the belly area of the child, and the area may swell and expand. Because of the lump in the abdomen, the child may not want to eat and may complain about having pain in the stomach.
Proptosis (eye protrusion) and ecchymosis (purple discoloration under the skin), if present, are due to the tumor depositing into the cavity of the skull where the eye rests.
Other problems may also occur. For example, the tumor may press against the bladder or bowel, causing urinary problems, constipation, or diarrhea.

Diagnosis

To diagnose ganglioneuroblastomas, the doctor may ask about the child's symptoms and run some tests. These tests include blood, urine, imaging, and bone scans.
Blood and urine samples can detect specific chemicals released by the tumor, such as lactate dehydrogenase, ferritin, dopamine, homovanillic acid, and vanillylmandelic acid.
Imaging tests, such as a chest X-ray (CXR), magnetic resonance imaging (MRI), or a computerized tomography (CT) scan, allow the doctor to see inside the body for the possible cancer and to see if it has spread to other parts of the body. These tests may also let the doctor see if the treatment is working against the cancer.
Bone scans, such as a metaiodobenzylguanidine (mIBG), may also be helpful for finding the cancer if there is bone involvement. mIBG has specificity and sensitivity for bone and bone marrow diseases.
If these tests show cancer, a biopsy may be performed, where a small portion of the cancer may be removed with a needle. The removed portion is then viewed under a microscope. This allows the doctor to be certain that it is indeed a ganglioneuroblastoma.
If a diagnosis is made, the tumor is then staged, based on the International Neuroblastoma Staging System (INSS). The stage of the tumor is based on respectability, local lymph node involvement, localization, and dissemination. Staging is done in order to tailor treatment and evaluate the prognosis.

Complications

Horner's syndrome: This typically occurs with tumors located in the cervix or in the thoracic (chest) region and involves only one side of the face. It is characterized by a drooping eyelid, a constricted pupil, and reduced facial sweat.
Hutchinson syndrome: This may occur when the tumor has metastasized (spread) to the bone, and it is characterized by limping and irritability.
Kerner-Morrison syndrome: Dehydration and hypokalemia (low potassium levels) may result. This is observed with tumors that secrete vasoactive intestinal polypeptides, hormones that cause the intestine to secrete water and electrolytes. Hypokalemia may cause arrhythmias (abnormal heart rhythms).
Opsoclonus-myoclonus-ataxia syndrome: This may occur due to the body producing antibodies against the tumor, and it manifests as uncontrolled jerking movements of the arms, legs, and chest, and rapid eye movements. Developmental delays in the child may result.
Spinal cord compression (SCC): This may occur in children when the tumor has spread to areas surrounding the spinal cord. Signs and symptoms of SCC include back pain, loss of sensation, numbness, stiffness in the arms or legs, and loss of bladder or bowel control.

Treatment

General: The treatment for ganglioneuroblastoma depends on the spread of the cancer, age, and other risk factors. Possible treatments include surgery, chemotherapy, radiation, stem cell transplant, and immunotherapy. Many children with ganglioneuroblastomas have a good chance of survival following proper treatment. Because the cancer can grow and spread, finding it early and beginning treatment right away are important. If a ganglioneuroblastoma is suspected, it is suggested to contact a doctor immediately.
Surgery: If the cancer has not spread, doctors may choose to take it out. Even if surgery is used, a child may still need chemotherapy or radiation therapy to make sure that all of the cancer is gone.
Chemotherapy: This treatment involves the use of drugs that are put directly into the blood so that they can reach the cancer cells and destroy them. While often useful, these drugs have some side effects, such as hair loss, nausea, vomiting, diarrhea, and fatigue. These agents are used in combination with each other and other therapies. Some agents used in the treatment of ganglioneuroblastomas include antineoplastic agents (cisplatin, carboplatin, doxorubicin, etoposide, cyclophosphamide, thiotepa, and vincristine), colony-stimulating factors (filgrastim), and chemoprotective agents (mesna).
Radiation therapy: This therapy uses high-energy beams to destroy cancer cells.
Stem cell transplant: This procedure helps replace bone marrow in the body. The body's bone marrow is the factory for new blood cells. Without bone marrow, dead cells cannot be replaced, and cells cannot be made to fight against disease. Unfortunately, treatments like chemotherapy and radiation may destroy bone marrow while they work to destroy the cancer. To fix this problem, a stem cell transplant may be used. Stem cells are special cells that make their way into the bone marrow and replace the good cells that were destroyed. Stem cells may be taken from the person receiving the treatment or from someone else.
Immunotherapy: This treatment uses the body's own immune system to fight the cancer. Immunotherapy is often used after other treatments, such as surgery, radiation therapy, or chemotherapy.

Integrative therapies

Note: Ganglioneuroblastomas are a serious medical condition that requires immediate medical treatment. Although some evidence suggests that certain integrative therapies may be helpful for alleviation of side effects of treatment of ganglioneuroblastomas, such as hair loss, nausea, vomiting, and fatigue, these therapies should not replace or delay treatment with more proven techniques or therapies for ganglioneuroblastomas.
Strong scientific evidence:
Acupressure: There is strong evidence from meta-analyses and randomized controlled trials to support the use of P6 (a specific point on the wrist) acupressure in the prevention and treatment of acute nausea and vomiting. These studies have found acupressure effective in alleviating postoperative nausea, intraoperative nausea (during spinal anesthesia), chemotherapy-induced nausea, and pregnancy-related nausea (morning sickness).
With proper training, acupressure appears to be safe if self-administered or administered by an experienced therapist. No serious long-term complications have been reported, according to scientific data.
Good scientific evidence:
Acupuncture: Evidence from several small studies supports the use of acupuncture at a specific point on the wrist (P6) to help chemotherapy patients reduce nausea and vomiting. Electroacupuncture has also been used in combination with antinausea medications.
Needles must be sterile in order to avoid disease transmission.
Unclear or conflicting scientific evidence:
Acustimulation: The evidence on the use of acustimulation wristbands for chemotherapy-related nausea and vomiting is mixed. Some studies suggest no benefit and that simple acupressure massage may be more effective. Other studies have found benefit, in that when acustimulation is combined with antinausea medications, the bands may reduce the amount of medication needed, and they may help with more severely nauseated patients. More studies are needed to clarify the relationship between patient responses, types of cancer, and types of chemotherapy.
The only known side effect of acustimulation devices is a slight skin irritation under the electrodes when the wristband is used. Switch wrists to avoid this.
Aromatherapy: It is unclear whether, or to what extent, aromatherapy might benefit quality of life in cancer patients, and whether it may have an additive effect when combined with regular massage. More studies are needed to evaluate whether aromatherapy contributes to improved quality of life in patients with cancer.
Aspartic acid: Evidence from clinical research suggests that potassium and magnesium salts of aspartate may have fatigue-reducing effects. However, the effects of aspartic acid alone are unclear. Secondary sources suggest that aspartates may be low in individuals with chronic fatigue syndrome. Additional clinical research is needed in this area.
Use cautiously in patients with high protein intakes, gastrointestinal concerns, liver damage, gout, kidney damage, or osteoporosis.
Bee pollen: Bee pollen taken by mouth may reduce some adverse effects of cancer treatment. These adverse effects include anorexia, nausea, hair loss, inflammation, and sleeplessness. Additional studies are needed before a firm recommendation can be made.
Avoid if allergic or hypersensitive to bee pollen or other bee products. Avoid with liver disease or bleeding disorders. Use cautiously, because bee pollen may contain mycotoxins, such as ochratoxin A. Avoid if pregnant or breastfeeding, due to a lack of scientific data.
Cedar: Cedar is native to the western Himalayan mountains and the Mediterranean region. In one clinical study, patients with alopecia areata who were massaged with a combination of cedarwood oil, other aromatic oils, and carrier oils had significantly improved symptoms.
Avoid if allergic or hypersensitive to cedar, its pollen, wood dust, constituents, or members of the Pinaceae family. Avoid if pregnant or breastfeeding.
Coenzyme Q10 (CoQ10): Early research shows that CoQ10 may improve symptoms of chronic fatigue syndrome. High-quality research is needed in this area before a decision can be made.
Stop use two weeks before surgery or dental or diagnostic procedures with a bleeding risk, and do not use immediately after these procedures. Use caution with a history of blood clots, diabetes, high blood pressure, heart attack, or stroke, or with anticoagulants (blood thinners) or antiplatelet drugs (like aspirin, warfarin, or clopidogrel (Plavix?)), blood pressure drugs, blood sugar drugs, cholesterol drugs, or thyroid drugs. Avoid if pregnant or breastfeeding.
DHEA: The scientific evidence remains unclear regarding the effects of dehydroepiandrosterone (DHEA) supplementation in patients with chronic fatigue syndrome. Better research is necessary before a clear conclusion can be drawn.
Avoid if allergic to DHEA products. Few side effects have been reported when DHEA supplements were taken by mouth in recommended doses.
Ginger: Preliminary evidence suggests that ginger may have benefits on decreased white blood cells due to chemotherapy. While the results are promising, the role of ginger is unclear, and additional studies are needed to make a firm conclusion. Initial human research reports that ginger may reduce the severity and length of time that cancer patients feel nauseated after chemotherapy. Other studies show no effects. Additional studies are needed to confirm these results and to determine safety and dosing. Numerous prescription drugs are highly effective at controlling nausea in cancer patients undergoing chemotherapy, and the available options should be discussed with the patient's oncologist.
Avoid if allergic to ginger or other members of the Zingiberaceaefamily (such as red ginger, Alpinia purpurata, shell ginger, Alpinia zeru, green cardamom, and balsam of Peru). Avoid with anticoagulation therapy (therapy aimed at preventing the blood from clotting).
Ginseng: A few studies using ginseng extract G115? (with or without multivitamins) reported improvements in patients with fatigue of various causes. However, these results are early, and the studies have not been of high quality.
Avoid ginseng with a known allergy to plants in the Araliaceae family. There has been a report of a serious life-threatening skin reaction, possibly caused by contaminants in ginseng formulations.
Hypnosis: There are several studies that used hypnosis for cancer-related conditions, including hot flashes, pain, anxiety, depression, difficulty sleeping, and quality of life. Limited research has reported mixed effects of hypnotherapy for chemotherapy-related nausea, vomiting, or mouth sores. Hypnosis during tumor treatment and removal has been shown to reduce pain, anxiety, and medication use. Additional research is necessary before a conclusion can be drawn.
Kefir: Although scientific evidence supporting the use of kefir, a probiotic made of bacteria, yeast, and carbohydrates, in chemotherapy patients is lacking, the evidence is preliminary, and well-designed studies are needed before conclusions may be drawn.
Caution is advised in patients with known allergy or hypersensitivity to dairy products.
Lavender: Lavender is grown around the world. Oils from the flowers are used in aromatherapy, baked goods, candles, cosmetics, detergents, jellies, massage oils, perfumes, powders, shampoo, soaps, and teas. Small studies have shown that patients who massage essential oils (thyme, rosemary, lavender, and cedarwood) into their scalps daily experienced an improvement in hair loss. More research on lavender alone is need before a firm conclusion can be made.
Avoid if allergic or hypersensitive to lavender. Avoid with a history of seizures, bleeding disorders, eating disorders (anorexia, bulimia), or anemia (low levels of iron). Avoid if pregnant or breastfeeding.
L-carnitine: There are several promising reports on the use of L-carnitine for fatigue. However, additional research is warranted in this area.
Avoid with known allergy or hypersensitivity to carnitine. Use cautiously with peripheral vascular disease, hypertension (high blood pressure), alcohol-induced liver cirrhosis, or diabetes. Use cautiously in low-birthweight infants and individuals on hemodialysis. Use cautiously if taking anticoagulants (blood thinners), beta-blockers, or calcium channel blockers. Avoid if pregnant or breastfeeding.
Music therapy: Music therapy has been investigated as a means of reducing levels of nausea and episodes of vomiting postoperatively or in patients receiving high-dose chemotherapy, with mixed results. However, further research is required before a firm conclusion can be drawn.
Music therapy is generally known to be safe.
Peppermint: Peppermint, a perennial herb growing to the size of one meter and found throughout much of Europe and North America, has a long history of use for digestive disorders. More research is needed to determine if peppermint inhalation is an effective treatment for postoperative nausea.
Avoid if allergic to peppermint oil or members of the Labiatae/Lamiaceae family. Avoid topical use of peppermint oil around the facial or chest areas of infants and young children, especially around the nose. Avoid injection of peppermint oil. Use cautiously in patients with gastroesophageal reflux disease, achlorhydria, G6PD deficiency, gallbladder disease, hiatal hernias, or kidney stones.
Scopolamine: There is insufficient evidence from available studies to support the use of scopolamine in controlling chemotherapy-induced emesis (vomiting). The potential role of transdermal scopolamine as adjunct therapy with other antiemetic drugs needs further testing.
Use cautiously in patients that are pregnant, trying to get pregnant, or breastfeeding. Use cautiously in patients with heart conditions, blood pressure disorders, psychiatric disorders, sensitive skin, eye disorders, autonomic neuropathy, or hyperthyroidism, and in patients with a history or seizures. Use cautiously with alcohol, before surgery, with other anticholinergic drugs, or with CNS depressants. Use cautiously in elderly patients and in scuba divers in deep water (below 50-60 feet). Avoid in patients with a known allergy or hypersensitivity to scopolamine, atropine, belladonna, hyoscyamine, anticholinergic drugs, or any component of the transdermal system. Avoid scopolamine patches during a magnetic resonance imaging (MRI) procedure. Avoid in patients with liver, kidney, or lung insufficiency, narrow-angle glaucoma, obstructive uropathy (for example, bladder neck obstruction due to prostatic hypertrophy), obstructive disease of the gastrointestinal tract (as in achalasia, pyloroduodenal stenosis), paralytic ileus, or intestinal atony of the elderly or debilitated, severe ulcerative colitis or toxic megacolon complicating ulcerative colitis, myasthenia gravis, or chronic lung disease. Avoid driving or using heavy machinery when using scopolamine, which may cause drowsiness and fatigue.
Rosemary: Rosemary (Rosmarinus officinalis L.) is a common evergreen aromatic shrub that is grown in many parts of the world. The fresh and dried leaves are used frequently in traditional Mediterranean foods as a flavoring agent and as a food preservative. Historically, rosemary has been used to stimulate the growth of hair. Rosemary oil has been shown to increase circulation and possibly promote hair growth in patients with alopecia areata. Additional research is warranted to confirm these findings.
Avoid if allergic or hypersensitive to rosemary, its constituents, or members of the Labiatae/Lamiaceae family. Use cautiously if prone to atopic or hypersensitivity reactions. Use cautiously with a history of iron deficiency anemia. Use cautiously with blood clotting disorders, low blood pressure, diabetes, or peptic ulcer disease. Use cautiously if taking blood thinners, agents used to lower blood pressure, anticancer drugs, medication for high cholesterol, herbs or drugs that are broken down by the liver, diuretics, estrogen or estrogenic herbs, or medications for osteoporosis. Avoid if taking lithium. Avoid if pregnant or breastfeeding.
TENS: Transcutaneous electrical nerve stimulation (TENS) is a noninvasive technique in which a low-voltage electrical current is delivered through wires from a small power unit to electrodes located on the skin. TENS is usually performed in conjunction with acupuncture treatments. Electrodes are temporarily attached with paste in various patterns, depending on the specific condition and treatment goals. Although limited available research found positive results when using TENS with postoperative nausea and vomiting, more research is needed. There is also not enough reliable evidence to draw a firm conclusion on the use of TENS for nausea and vomiting related to pregnancy.
Avoid with implantable devices, such as defibrillators, pacemakers, intravenous infusion pumps, or hepatic artery infusion pumps. Use cautiously with decreased sensation (such as neuropathy) or with seizure disorders. Avoid if pregnant or breastfeeding.
Thyme: Thyme is a common herb used in cooking. It has also been used for thousands of years to treat medical conditions. Thyme has been suggested as a possible treatment for hair loss. However, further research is needed to confirm these claims.
Avoid if allergic or hypersensitive to thyme, constituents of thyme, members of the Labiatae/Lamiaceae (mint) family, or rosemary (Rosmarinus officinalis). Avoid oral ingestion or nondiluted topical application of thyme oil, due to potential toxicity. Avoid topical preparations in areas of skin breakdown or injury or in atopic patients, due to multiple reports of contact dermatitis. Use cautiously with gastrointestinal irritation or peptic ulcer disease, due to anecdotal reports of gastrointestinal irritation. Use cautiously with thyroid disorders, due to observed antithyrotropic effects in animal research on the related species Thymus serpyllum. Avoid if pregnant or breastfeeding.
Thymus extract: Preliminary evidence suggests that thymus extract may be useful for alopecia or hair regrowth. More clinical trials are required before recommendations can be made involving thymus extract for this use.
Avoid if allergic or hypersensitive to thymus extracts. Use bovine thymus extract supplements cautiously due to the potential for exposure to the virus that causes "mad cow disease." Avoid use with an organ transplant or other forms of allografts or xenografts. Avoid with thymic tumors, myasthenia gravis (a neuromuscular disorder), or untreated hypothyroidism. Avoid if receiving immunosuppressants or hormonal therapy. Avoid if pregnant or breastfeeding. Thymic extract increases human sperm motility and progression.
Vitamin A: The effect of vitamin A supplementation on chemotherapy-related side effects, including nausea and vomiting, is unclear. Also, it is unclear if vitamin A interacts with chemotherapy agents. Further research is needed.
Vitamin A toxicity can occur if taken at high dosages. Vitamin A appears to be safe in pregnant women if taken in recommended doses. However, vitamin A excess, as well as deficiency, has been associated with birth defects. Vitamin A is excreted in human breast milk. The benefits or dangers to nursing infants are not clearly established. Use cautiously in children and infants. Use cautiously in patients using alcohol, bile acid sequestrants, birth control pills, chemotherapeutic agents (such as busulfan), mineral oil, neomycin, or orlistat. Avoid if allergic or hypersensitive to vitamin A. Avoid with agents toxic to the liver, blood thinners (such as warfarin (Coumadin?)), retinoids, or tetracycline antibiotics. Avoid in patients with liver disease, intestinal infections, fat-malabsorption syndromes, severe protein energy malnutrition, or type V hyperlipoproteinemia (a genetic disorder). Avoid high-dose vitamin A and beta-carotene in patients at high risk of lung cancer.
Vitamin B12: There is some evidence that intramuscular injections of vitamin B12 given twice per week may improve the general well-being and happiness of patients with symptoms of tiredness or fatigue. However, fatigue has many potential causes, and well-designed clinical trials are needed before a recommendation can be made.
Avoid if allergic or hypersensitive to cobalamin, cobalt, or any other vitamin B12 product ingredients. Avoid with coronary stents (mesh tubes that hold clogged arteries open) and Leber's disease. Use cautiously if undergoing angioplasty and with anemia. Vitamin B12 is generally considered safe when taken in amounts that are not higher than the recommended dietary allowance (RDA). There are not enough scientific data available about the safety of larger amounts of vitamin B12 during pregnancy or breastfeeding.
Yoga: Preliminary studies in humans report that yoga may improve fatigue in adults. However, better-designed studies are needed before any conclusion can be made.
Yoga is generally considered to be safe in healthy individuals when practiced appropriately. Avoid some inverted poses with disc disease of the spine, fragile or atherosclerotic neck arteries, risk for blood clots, extremely high or low blood pressure, glaucoma, detachment of the retina, ear problems, severe osteoporosis, or cervical spondylitis. Certain yoga breathing techniques should be avoided in people with heart or lung disease. Use cautiously with a history of psychotic disorders. Yoga techniques are believed to be safe during pregnancy and breastfeeding when practiced under the guidance of expert instruction (the popular Lamaze techniques are based on yogic breathing). However, poses that put pressure on the uterus, such as abdominal twists, should be avoided during pregnancy.
Traditional or theoretical uses lacking sufficient evidence:
Chamomile: Chamomile has been suggested as potential treatment for nausea and vomiting. However, high-quality human evidence is currently lacking.
Avoid if allergic to chamomile. Anaphylaxis (severe allergic reaction), throat swelling, skin allergic reactions, and shortness of breath have been reported. Chamomile eyewash may cause allergic conjunctivitis (pinkeye). Stop use two weeks before and immediately after surgery or dental or diagnostic procedures with bleeding risks. Use cautiously if driving or operating machinery. Avoid if pregnant or breastfeeding.
Lavender: Although lavender has been suggested as a potential treatment for nausea and vomiting, high-quality human evidence is currently lacking.
Avoid if allergic or hypersensitive to lavender. Avoid with a history of seizures, bleeding disorders, eating disorders (anorexia, bulimia), or anemia (low levels of iron). Avoid if pregnant or breastfeeding.

Prevention

High-quality clinical data on the prevention of ganglioneuroblastomas are currently lacking.

Author information

This information has been edited and peer-reviewed by contributors to the Natural Standard Research Collaboration (www.naturalstandard.com).

Bibliography

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American Cancer Society. .
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Causes

The cause of ganglioneuroblastomas is unknown, but age and genetics are thought to play a role.
Ganglioneuroblastomas may be linked to environmental factors, such as fetal exposure to pesticides, alcohol, or phenobarbital, and assisted pregnancies, but none have been confirmed.
There may be a genetic predisposition to developing ganglioneuroblastomas in those with Hirschsprung disease, neurofibromatosis type 1, congenital central hypoventilation, and von Recklinghausen disease. Chromosome abnormalities involving chromosome 1 may also be linked to the development of ganglioneuroblastomas.

Types of the disease

Intermixed ganglioneuroblastoma: This comprises well-defined neuroblastic cells (cell that will develop into neurons) that are at various stages of differentiation, distributed in the tumor at random.
Nodular ganglioneuroblastoma: This contains highly visible nodules that are usually hemorrhagic (bleeding) and are in the same space with ganglioneuroblastoma intermixed cells.