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Ganglioneuroma is a tumor of the peripheral nervous system.
Causes, incidence, and risk factorsGanglioneuromas are rare tumors that most frequently start in the autonomic nerve cells, which may be in any part of the body. The tumor are usually noncancerous (benign).
Ganglioneuromas usually occur in people ages 10 to 40. They grow slowly, and may release certain chemicals or hormones.
There are no known risk factors. However, the tumors may be associated with some genetic problems, such as neurofibromatosis type 1.
SymptomsA ganglioneuroma usually causes no symptoms, and is only discovered when being examined or treated for another condition.
Symptoms depend on the location of the tumor and the type of chemicals released.
If the tumor is in the chest area (mediastinum), symptoms may include:
- Breathing difficulty
- Chest pain
- Compression of the windpipe (trachea)
If the tumor is lower down in the abdomen in the area called the retroperitoneal space, symptoms may include:
- Abdominal pain
- Bloating
If the tumor is near the spinal cord, it may cause:
- Compression of the spinal cord, which leads to pain and loss of strength or feeling in the legs, the arms, or both
- Spine deformity
These tumors may produce certain hormones, which can cause the following symptoms:
- Diarrhea
- Enlarged clitoris (women)
- High blood pressure
- Increased body hair
- Sweating
The best tools to identify a ganglioneuroma are:
Blood and urine tests may be done to determine if the tumor is producing hormones or other chemicals.
A biopsy or complete removal of the tumor may be needed to confirm the diagnosis.
TreatmentTreatment involves surgery to remove the tumor (if it is causing symptoms).
Expectations (prognosis)Most ganglioneuromas are noncancerous. The expected outcome is usually good. A ganglioneuroma may, however, become cancerous and spread to other areas, or it may come back after removal.
ComplicationsIf the tumor has been present for a long time and has pressed on the spinal cord or caused other symptoms, surgery to remove the tumor may not necessarily reverse the damage.
Compression of the spinal cord may result in loss of movement (paralysis), especially if the cause is not detected promptly.
Surgery to remove the tumor may also lead to complications in some cases.
Calling your health care providerCall your health care provider if you or your child has symptoms that may be caused by this type of tumor.
ReferencesSovak MA, Aisner SC, Aisner J. Tumors of the pleura and mediastinum. In: Abeloff MD, Armitage JO, Niederhuber JE, Kastan MB, McKenna WG, eds.Clinical Oncology. 4th ed. Philadelphia, Pa: Churchill Livingstone Elsevier; 2008:chap 77.
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Ganglioneuroblastoma?
DefinitionGanglioneuroblastoma is an intermediate tumor arising from nerve tissue. An intermediate tumor is one that is between benign (slow-growing and unlikely to spread) and malignant (fast-growing, aggressive, and likely to spread).Causes, incidence, and risk factorsThis rare tumor has a yearly occurrence of less than 5 per 1,000,000 children.Tumors of the nervous system vary in their degree of differentiation. The degree of differentiation determines how the tumors appear under the microscope and whether or not they are likely to spread.Benign tumors are less likely to spread. Malignant tumors are aggressive, grow quickly, and often spread. A ganglioneuroma is a benign tumor, while a neuroblastoma (occurring in children more than a year old) is generally malignant.A ganglioneuroblastoma may be localized to one area or it may be widespread, but it is usually less aggressive than a neuroblastoma. The cause is unknown.SymptomsMost commonly, a mass can be felt in the abdomen, but this condition may also occur in other parts of the body.Signs and testsBone marrow aspiration and biopsy may be necessary.Bone scan may be necessary.CT scan or MRI scan of the affected areaMIBG scan may be necessary.Specialized blood and urine testsSurgical biopsy to confirm diagnosisTreatmentBecause these tumors are rare, they should be treated in a specialized center by experts who have experience with them.Depending on the specific nature of the tumor, treatment can consist of surgery, and possibly chemotherapy and radiation therapy.Support GroupsThe stress of illness can often be helped by joining a support group where members share common experiences and problems. See cancer - support group.Expectations (prognosis)The prognosis depends on the extent of the tumor and whether or not some areas of the tumor contain the more aggressive cells of a neuroblastoma.ComplicationsInvasion of the tumor into surrounding areas (spread of the tumor)Complications of surgery, radiation, or chemotherapyCalling your health care providerCall your health care provider if you feel a mass or growth on your child's body. Make sure children receive routine examinations as part of their well child care.ReferencesSovak MA, Aisner SC, Aisner J. Tumors of the pleura and mediastinum. In: Abeloff MD, Armitage JO, Niederhuber JE, Kastan MB, McKenna WG, eds. Abeloff's Clinical Oncology. 4th ed. Philadelphia, Pa: Elsevier Churchill Livingstone; 2008:chap 77.Kim S, Chung DH. Pediatric solid malignancies: neuroblastoma and Wilms' tumor. Surg Clin North Am. 2006;86(2):469-487.Park JR, Eggert A, Caron H. Neuroblastoma: biology, prognosis, and treatment. Pediatr Clin North Am. 2008;55(1):97-120.
Catecholamines - blood?
DefinitionCatecholamines are hormones produced by the adrenal glands, which are found on top of the kidneys. They are released into the blood during times of physical or emotional stress. The major catecholamines are dopamine, norepinephrine, and epinephrine (which used to be called adrenalin).This article discusses the test to check the level of catecholamines in a sample of blood.Catecholamines are more often measured with a urine test than with a blood test. See: Catecholamines - urineAlternative NamesNorepinephrine - blood; Epinephrine - blood; Adrenalin - blood; Dopamine - bloodHow the test is performedBlood is typically drawn from a vein, usually from the inside of the elbow or the back of the hand. The site is cleaned with germ-killing medicine (antiseptic). The health care provider wraps an elastic band around the upper arm to apply pressure to the area and make the vein swell with blood.Next, the health care provider gently inserts a needle into the vein. The blood collects into an airtight vial or tube attached to the needle. The elastic band is removed from your arm.Once the blood has been collected, the needle is removed, and the puncture site is covered to stop any bleeding.In infants or young children, a sharp tool called a lancet may be used to puncture the skin and make it bleed. The blood collects into a small glass tube called a pipette, or onto a slide or test strip. A bandage may be placed over the area if there is any bleeding.How to prepare for the testThe accuracy of the test can be affected by certain foods and drugs, as well as physical activity and stress.Foods that can increase catecholamine levels include:CoffeeTeaBananasChocolateCocoaCitrus fruitsVanillaYou should avoid these foods for several days prior to the test, particularly if both blood and urine catecholamines are to be measured.You should also avoid stressful situations and vigorous exercise, which can both interfere with test results.Drugs that can increase catecholamine measurements include:AminophyllineCaffeineChloral hydrateClonidineDisulfiramErythromycinInsulinLevodopaLithiumMethenamineMethyldopaNicotinic acid(large doses)NitroglycerinQuinidineTetracyclineDrugs that can decrease catecholamine measurements include:ClonidineDisulfiramGuanethidineImipramineMAO inhibitorsPhenothiazinesReserpineSalicylatesNever stop taking any medication without first talking to your doctor.How the test will feelSome people feel discomfort when the needle is inserted. Others may notice only a prick or stinging sensation. Afterward, there may be some throbbing.Why the test is performedThis test is used to diagnose or rule out a pheochromocytoma or neuroblastoma. It may also be done in patients with those conditions to determine if treatment is working.Normal ValuesEpinephrine: 0-900 picograms/milliliter (pg/ml)Norepinephrine: 0-600 pg/mlNote: Normal value ranges may vary slightly among different laboratories. Talk to your doctor about the meaning of your specific test results.What abnormal results meanHigher-than-normal levels of blood catecholamines may suggest:Acute anxietyGanglioblastoma(very rare)Ganglioneuroma(very rare)Neuroblastoma(rare)Pheochromocytoma(rare)Severe stressAdditional conditions under which the test may be performed include Shy-Drager syndrome.What the risks areThere is very little risk involved with having your blood taken. Veins and arteries vary in size from one patient to another and from one side of the body to the other. Taking blood from some people may be more difficult than from others.Other risks associated with having blood drawn are slight but may include:Excessive bleedingFainting or feeling light-headedHematoma (blood accumulating under the skin)Infection (a slight risk any time the skin is broken)ReferencesYoung WF Jr. Adrenal medulla, catecholamines, and pheochromocytoma. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 246.Ferri FF. Laboratory tests and interpretation of results. In: Ferri FF, ed. Ferri's Clinical Advisor 2008: Instant Diagnosis and Treatment. 1st ed. Philadelphia, Pa: Mosby Elsevier; 2008:section IV.
Neuroblastoma?
DefinitionNeuroblastoma is a malignant (cancerous) tumor that develops from nerve tissue. It occurs in infants and children.Causes, incidence, and risk factorsNeuroblastoma can occur in many areas of the body. It develops from the tissues that form the sympathetic nervous system (the part of the nervous system that controls body functions, such as heart rate and blood pressure, digestion, and levels of certain hormones).Most neuroblastomas begin in the abdomen in the adrenal gland or next to the spinal cord, or in the chest. They may also start in other areas. Neuroblastomas can spread to the bones (face, skull, pelvis, shoulders, arms, and legs), bone marrow, liver, lymph nodes, skin, and around the eyes (orbits).The cause of the tumor is unknown. Neuroblastoma is most commonly diagnosed in children before age 5. The disorder occurs in approximately 1 out of 100,000 children and is slightly more common in boys.In most patients, the neuroblastoma has already spread when it is first diagnosed.SymptomsThe first symptoms are usually fever, a general sick feeling (malaise), and pain. There may also be loss of appetite, weight loss, and diarrhea.Other symptoms depend on the site of the tumor, and may include:Bone pain or tenderness (if the cancer spreads to the bones)Difficulty breathing or a chronic cough (if the cancer spreads to the chest)Enlarged abdomen (from a large tumor or excess fluid)Flushed, red skinPale skin and bluish color around the eyesProfuse sweatingRapid pulse (tachycardia)Brain and nervous system problems may include:Inability to empty the bladderLoss of movement (paralysis) of the hips, legs, or feet (lower extremities)Problems with balanceUncontrolled eye movements or leg and feet movements (called opsoclonus-myoconus syndrome, or "dancing eyes and dancing feet")Signs and testsSigns vary depending on the location of the tumor. Examination of the abdomen with the hands (palpation) may reveal a mass. The liver may be enlarged, if the tumor has spread to the liver. Adrenal gland tumors can cause high blood pressure and a fast heart rate. Lymph nodes may be swollen.X-ray or imaging tests are done to locate the main (primary) tumor and to see where it has spread. These include:Bone scanBone x-raysChest x-rayCT scan of chest and abdomenMRI scan of chest and abdomenOther tests include:Biopsy of tumorBone marrow biopsyCBC showing anemia or other abnormalityCoagulation studies, erythrocyte sedimentation rateHormone tests (blood tests to check levels of hormones such as epinephrine and other catecholamines)MIBG scanUrine 24-hour test for catecholamines, homovanillic acid (HVA) and vanillymandelic acid (VMA)TreatmentTreatment varies depending on the location of the tumor, how much and where the tumor has spread, and the age of the patient. In certain cases, surgery alone is enough, but often other therapies are needed. Anticancer medications (chemotherapy) may be recommended if the tumor is widespread. Radiation therapy may also be used.Support GroupsThe stress of illness can often be helped by joining a support group where members share common experiences and problems. See cancer - support group.Expectations (prognosis)The expected outcome varies. In very young children with neuroblastoma, the tumor may go away on its own, without any treatment, or the tissues of the tumor may mature and develop into a benign (non-cancerous) tumor called a ganglioneuroma, which can be surgically removed. In other cases, the tumor spreads rapidly.Response to treatment also varies. Treatment is often successful if the cancer has not spread, but if it has been spread, neuroblastoma is much harder to cure. Younger children often do better than older children.Tumors with certain genetic characteristics may be harder to cure.Children treated for neuroblastoma may be at risk for getting a second, different cancer in the future.ComplicationsSpread (metastasis) of the tumorDamage and loss of function of involved organ(s) Kidney failureLiver failureLoss of blood cells produced by the bone marrowDecreased resistance to infectionOther organ system failuresCalling your health care providerCall your health care provider if your child has symptoms of neuroblastoma. Early diagnosis and treatment improves the chance of a good outcome.ReferencesDome JS, Rodriguez-Galindo C, Spunt SL, Santana VM. Pediatric Solid Tumors. In: Abeloff MD, Armitage JO, Niederhuber JE, Kastan MB, McKenna WG, eds. Clinical Oncology. 4th ed. Philadelphia, Pa; Churchill Livingstone Elsevier; 2008: chap 99.
Catecholamines - urine?
DefinitionCatecholamines are small molecules made by nerve tissue (including the brain) and the adrenal gland.The major catecholamines are dopamine, norepinephrine, and epinephrine. These substances break down into other compounds, which leave your body through your urine.A urine test can be done to measure the level of catecholamines in your body.Catecholamines can also be measured with a blood test. See also: Catecholamines - bloodAlternative NamesDopamine-urine test; Epinephrine-urine test; Adrenalin-urine test; Vanillylmandelic acid (VMA); Urine metanephrine; Normetanephrine; Norepinephrine-urine test; Urine catecholamines; VMA; HVA; Metanephrine; Homovanillic acid (HVA)How the test is performedFor this test, you must urinate into a special bag or container every time you use the bathroom for 24-hour period.On day 1, urinate over the toilet into the container or bag when you wake up in the morning. Close the container tightly. Keep it in the refrigerator or a cool place during the collection period.Urinate into the special container every time you use the bathroom for the next 24 hours.On day 2, urinate into the container in the morning again when you wake up.Label the container with your name, the date, the time of completion, and return it as instructed.For an infant:Thoroughly wash the area around the urethra (the hole where urine flows out). Open a urine collection bag (a plastic bag with an adhesive paper on one end).For males, place the entire penis in the bag and attach the adhesive to the skin.For females, place the bag over the two folds of skin on either side of the vagina (labia). Put a diaper on the baby (over the bag).Check the infant frequently, and change the bag after the infant has urinated. Empty the urine from the bag into the container provided by your doctor.Because lively infants can cause the bag to move, this procedure may take a couple of attempts. You may need extra collection bags.When finished, label the container and return it as instructed.How to prepare for the testAcute stress and vigorous exercise may affect the test results.Foods that can increase urinary catecholamines include coffee, tea, bananas, chocolate, cocoa, citrus fruits, and vanilla. Avoid these foods for several days prior to the test.Certain drugs can also affect test results. Your health care provider may tell you to stop taking certain medicines before the test. Never stop taking medicine without first talking to your doctor.The following drugs can increase catecholamine measurements:Acetaminophen (Tylenol)AminophyllineCaffeineChloral hydrateClonidineDisulfiramErythromycinInsulinLevodopaLithiumMethenamineMethyldopaNicotinic acid(large doses)NitroglycerinQuinidineTetracyclinesDrugs that can decrease catecholamine measurements include:ClonidineDisulfiramGuanethidineImipramineMAO inhibitorsPhenothiazinesReserpineSalicylatesHow the test will feelThe test involves only normal urination, and there is no discomfort.Why the test is performedThe test is usually done to diagnose an adrenal gland tumor called pheochromocytoma. It may also be used to diagnose neuroblastoma. Urine catecholamine levels are increased in most persons with neuroblastoma.The urine test for catecholamines may also be used to monitor those who are receiving treatment for these conditions.Normal ValuesAll of the catecholamines are broken down into inactive substances that appear in the urine:Dopamine becomes Homovanillic acid (HVA)Norepinephrine becomes normetanephrine and vanillylmandelic acid (VMA)Epinephrine becomes metanephrine and VMAThe following normal values represent the amount of the substance found in the urine over a 24 hour period. Normal values vary from lab to lab, but in general are as follows:Dopamine: 65 - 400 micrograms (mcg)/24 hoursEpinephrine: 0.5 - 20 mcg/24 hoursMetanephrine: 24 - 96 mcg/24 hours (some laboratories give the range as 140 - 785 mcg/24-hours)Norepinephrine: 15 - 80 mcg/24 hoursNormetanephrine: 75 - 375 mcg/24 hoursTotal urine catecholamines: 14 - 110 mcg/24 hoursVMA: 2 - 7 milligrams (mg)/24 hoursWhat abnormal results meanElevated levels of urinary catecholamines may indicate:Acute anxietyGanglioblastoma(very rare)Ganglioneuroma(very rare)Neuroblastoma(rare)Pheochromocytoma(rare)Severe stressAdditional conditions under which the test may be performed:Multiple endocrine neoplasia (MEN) IIWhat the risks areThere are no risks.Special considerationsSeveral foods and drugs, as well as physical activity and stress, can affect the accuracy of this test.ReferencesYoung WF. Adrenal medulla, catecholamines, and pheochromocytoma. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 246.
