Pheochromocytoma

Chromaffin tumors; Paraganglionoma Pheochromocytoma is a rare tumor of adrenal gland tissue. It results in the release of too much epinephrine and norepinephrine, hormones that control heart rate, metabolism, and blood pressure . A pheochromocytoma (fe-o-kro-mo-si-TOE-muh) is a rare, usually noncancerous (benign) tumor that develops in the core of an adrenal gland. You have two adrenal glands, one just above each of your kidneys. Your adrenal glands produce hormones that give instructions to virtually every organ and tissue in your body. If you have a pheochromocytoma, your adrenal glands can produce too much of certain hormones, raising your blood pressure and heart rate. A pheochromocytoma may be life-threatening if unrecognized or untreated. A pheochromocytoma can develop at any age, but most commonly occurs in middle age. Usually, treatment for pheochromocytoma can return blood pressure to normal. Sometimes pheochromocytoma is part of another condition called multiple endocrine neoplasia syndrome (MEN). People with MEN often have other cancers and other problems involving hormones.

Causes, incidence, and risk factors

Pheochromocytoma may occur as a single tumor or as more than one growth. It usually develops in the center (medulla) of one or both adrenal glands. Rarely, this kind of tumor occurs outside the adrenal gland, usually somewhere else in the abdomen. Very few pheochromocytomas are cancerous. The tumors may occur at any age, but they are most common from early to mid-adulthood. CAUSE Researchers don't know exactly what causes cells within the adrenal gland to develop into a tumor. What they do know is that pheochromocytomas begin in special cells called chromaffin cells, which are found in the core of the adrenal gland. Your adrenal glands are part of your endocrine system, which produces hormones that regulate processes throughout your body. The role of hormones Your adrenal glands are located on top of your kidneys, and they produce hormones, including catecholamines. The most important of these are adrenaline (epinephrine) and noradrenaline (norepinephrine). Your body needs these hormones to maintain blood pressure and to help cope with stressful situations. Physical and emotional stresses usually trigger their release. When secreted into your bloodstream, catecholamines increase your heart rate and blood pressure and affect several other body functions. Multiple tumors possible Pheochromocytomas usually affect only one adrenal gland. However, you may have more than one tumor in an adrenal gland or you may develop tumors in both adrenal glands. Because chromaffin cells also are situated in nerve tissue throughout your body, pheochromocytomas occasionally arise outside of the adrenal glands (paraganglioma). Common locations for paraganglioma include the heart, neck, bladder, back wall of the abdomen and along the spine.

Causes of hypertensive crisis associated with pheochromocytoma An attack of high blood pressure (hypertensive crisis) associated with pheochromocytoma typically lasts less than an hour. Blood pressure may be normal or elevated between the episodic symptoms. Hypertensive crisis and other signs and symptoms may be brought on by emotional distress or anxiety, surgical anesthesia, or physical activities that press on the tumor, such as:

Changes in body position Exercise Pregnancy Lifting Having a bowel movement Urination

Additionally, these factors can trigger a high blood pressure episode:

Using drugs or stimulants that raise your blood pressure, such as decongestants, amphetamines or cocaine. Eating foods rich in tyramine an amino acid normally found in your body that helps regulate blood pressure. Tyramine is found in imported beer, some wines, processed meats or fish, fermented cheeses, soy sauce, bananas, avocados, and overripe or spoiled food. Taking a monoamine oxidase inhibitor (MAOI) to treat depression. Examples of MAOIs include phenelzine (Nardil), tranylcypromine (Parnate) and isocarboxazid (Marplan)

RISK FACTOR In some cases, pheochromocytomas run in families or are associated with an inherited condition, such as:

Multiple endocrine neoplasia, type II (MEN II). In addition to a pheochromocytoma, people with MEN II also have a rare form of thyroid cancer called medullary thyroid cancer. There are two subsets of MEN II, which include pheochromocytoma with medullary thyroid cancer and hyperparathyroidism (MEN IIA), and pheochromocytoma with medullary thyroid cancer and tumors of nerves in the lips, mouth, eyes and digestive tract (MEN IIB). Von Hippel-Lindau disease. People with this rare multisystem disorder are at high risk of pheochromocytoma. Neurofibromatosis 1 (NF1). Pheochromocytomas sometimes occur in people with NF1, a syndrome that includes multiple tumors in the skin (neurofibromas), pigmented skin spots and tumors of the optic nerve.

Most pheochromocytomas are noncancerous (benign) and don't spread to other parts of your body. However, cancerous (malignant) pheochromocytomas can spread throughout your body, including your brain, lungs or bone.

Symptoms

Abdominal pain Chest pain Irritability Nervousness Pallor Palpitations Rapid heart rate

Severe headache Profound Sweating Weight loss Feeling of anxiety High BP Feel/ extreme fright

Other symptoms that can occur with this disease:

Hand tremor High blood pressure Sleeping difficulty

Symptoms occur in discrete attacks at unpredictable intervals and usually last 15 to 20 minutes. The attacks may increase in frequency, length, and severity as the tumor grows. High blood pressure may occur only from time to time. These signs and symptoms develop because this type of tumor produces an excess of chemical compounds called catecholamines. Excessive secretion of catecholamines the hormones adrenaline (epinephrine) and noradrenaline (norepinephrine) can lead to persistent high blood pressure or wild fluctuations in your blood pressure, depending on whether the catecholamines are released continuously or in shorter bursts. The intermittent release of these hormones can cause other symptoms to occur from time to time as well. Although high blood pressure is a common sign of a pheochromocytoma, most people with high blood pressure don't have a pheochromocytoma. For some people, imaging studies, such as CT scans, done for another reason, may catch a pheochromocytoma before it produces symptoms. You may not have signs or symptoms if your pheochromocytoma is associated with a genetic condition, such as multiple endocrine neoplasia, type II (MEN II), because it's likely to be found on a scan before symptoms start.

Signs and tests

The doctor will perform a physical exam. You may have high blood pressure, rapid heart rate, and fever during an attack of symptoms. Your vital signs can be normal at other times. Tests include:

Abdominal CT scan Adrenal biopsy Catecholamines blood test Glucose test Metanephrine blood test MIBG scintiscan MRI of abdomen Urine catecholamines Urine metanephrines

Your doctor will likely conduct a number of tests to determine if a pheochromocytoma is the cause of your signs and symptoms. These may include:

Blood and urine tests. These tests can reveal elevated levels of the hormones adrenaline (epinephrine) and noradrenaline (norepinephrine) and their breakdown products (metanephrines).

You may need to collect urine samples over a 24-hour period for testing. If the results of these tests indicate a pheochromocytoma, the next step is to determine the location of the tumor. Imaging scans. A computerized tomography (CT) scan of your abdomen can detect the tumor in most cases. However, it may be necessary to scan other areas of your body, such as your neck, chest and pelvis. Scanning with other diagnostic imaging techniques, such as magnetic resonance imaging (MRI), a metaiodobenzylguanidine (MIBG) scan or a positron emission tomography (PET) scan, may be done to detect the tumor.

Incidental discovery Some adrenal gland tumors are discovered incidentally during imaging studies conducted for other reasons. For example, you might see your doctor because of abdominal pain, and a CT scan may reveal a mass on one of your adrenal glands. A small percentage of adrenal gland tumors found incidentally are pheochromocytomas, even when no signs or symptoms are present. If you have an adrenal mass found incidentally, your doctor will obtain blood and urine tests to see if there's hormone overproduction. Most small adrenal gland tumors are not pheochromocytomas and are noncancerous (benign), requiring only observation and no treatment. Genetic testing Genetic mutations may be responsible for pheochromocytoma and paraganglioma, and your doctor may suggest genetic testing. However, whether you need genetic testing and, if so, which type, depends on the location of your tumor and related medical conditions. If you have a history of pheochromocytoma or an associated disease, seek genetic counseling to help you decide if you should undergo genetic testing.

Treatment
Treatment involves removing the tumor with surgery. It is important to stabilize blood pressure and pulse with medication before surgery. You may need to stay in the hospital with close monitoring of your vital signs. After surgery, it is necessary to continually monitor all vital signs in an intensive care unit. When the tumor cannot be surgically removed, medication is needed to manage it. This usually requires a combination of medications to control the effects of the excessive hormones. Radiation therapy and chemotherapy have not been effective in curing this kind of tumor. The best treatment for most pheochromocytomas is surgery to remove the tumor. If you can't have the surgery or to lower your blood pressure before surgery, your doctor likely will prescribe drugs. Medications Medications used to treat high blood pressure associated with pheochromocytomas include:

Alpha blockers. Alpha blockers, also called alpha-adrenergic blocking agents or alpha-adrenergic antagonists, relax certain muscles that help small blood vessels remain open. They work by keeping the hormone noradrenaline (norepinephrine) from stimulating the muscles in the walls of smaller arteries and veins. This stimulation makes the vessel walls constrict. Blocking that effect causes the vessels to remain open and relaxed. This improves blood flow and lowers blood pressure. Examples of alpha blockers include phenoxybenzamine (Dibenzyline), doxazosin (Cardura), prazosin (Minipress) and terazosin (Hytrin). Side effects may include headache, pounding heartbeat, nausea and weight gain. Beta blockers. Beta blockers, also known as beta-adrenergic blocking agents, make your heart beat more slowly and with less force. They work by blocking the effects of the hormone norepinephrine. This action slows down the nerve impulses that travel through your heart. That means your heart doesn't pump as hard because it needs less oxygen and blood. Beta blockers also slow down the release of the enzyme renin from your kidneys, helping keep blood vessels dilated. Examples of beta

blockers include atenolol (Tenormin), metoprolol (Lopressor, Toprol XL) and propranolol (Inderal LA, Innopran XL). Side effects may include fatigue, headache, upset stomach and dizziness. Calcium channel blockers. Calcium channel blockers, also called calcium antagonists, relax and widen blood vessels in your arteries. They lower your blood pressure by preventing calcium from entering the cells of your heart and blood vessel walls. Examples of calcium channel blockers include amlodipine (Norvasc), diltiazem (Cardizem LA, Tiazac, others) and nicardipine (Cardene SR). Side effects may include constipation, headache, rapid heartbeat (tachycardia) and drowsiness. Metyrosine (Demser). This drug lowers blood pressure by inhibiting the production of catecholamines. It may be used when other drugs haven't worked or with other drugs. Side effects may include drowsiness, depression, anxiety and diarrhea.

For a pheochromocytoma, alpha blockers are used first to return your blood pressure back to within normal limits. Once your blood pressure is lowered, beta blockers can help control a rapid or irregular heartbeat. Any of these drugs may be used in preparation for surgery. Surgery to remove an adrenal gland The most common treatment for a pheochromocytoma is surgical removal of the entire affected adrenal gland. In most cases, signs and symptoms then disappear. Blood pressure usually returns to normal soon after surgery. Before surgery, your doctor will prescribe medications to block the effects of the adrenal hormones and control blood pressure. If both adrenal glands are affected by pheochromocytoma and are surgically removed, you'll need to take medication to replace the other hormones once produced by these glands. Laparoscopic surgery an option Depending on the size and location of the tumor, laparoscopic surgery may be performed. Laparoscopic surgery involves inserting instruments through several small incisions. This procedure may result in quicker recovery compared with conventional surgery, which requires a larger incision. Although it's becoming more widely used for pheochromocytoma, laparoscopic surgery isn't for everyone. Talk to your doctor about this less invasive technique to see if it's an option for you. Surgery may not be possible Sometimes surgery isn't an option because of the way the tumor is growing or because the tumor has spread (metastasized) to other parts of your body. If a pheochromocytoma is cancerous (malignant), treatment may go beyond medication and surgery to include radiation, chemotherapy or destroying the function (ablation) of arteries that supply blood to the tumor.

Expectations (prognosis)
Most patients who have noncancerous tumors that are removed with surgery are still alive after 5 years. The tumors come back in less than 10% of these patients. Levels of the hormones norepinephrine and epinephrine return to normal after surgery.

Complications
High blood pressure may continue in about 1 in 4 patients after surgery. However, standard treatments can usually control high blood pressure. In about 1 in 10 people, the tumor may return. Patients who have been successfully treated for pheochromocytoma should have testing from time to time to make sure the tumor hasn't returned. Close family members may also benefit from testing, depending on the exact type of tumor. If you have high blood pressure caused by a pheochromocytoma, excessive force on your artery walls can seriously damage many of your vital organs. The higher your blood pressure or the longer it goes uncontrolled, the greater the damage. Untreated high blood pressure may lead to:

Heart failure Stroke Kidney failure Acute respiratory distress Confusion Psychosis Seizures Visual impairment Premature death

A severe increase in blood pressure (hypertensive crisis) may occur with sudden release of a large amount of adrenaline hormones from the tumor. Any reading above 180/110 millimeters of mercury (mm Hg) is considered severe high blood pressure. This can lead to life-threatening conditions, such as stroke or abnormal heart rhythm (heart arrhythmia). Long-term exposure to these hormones can cause:

Damage to your heart muscle Congestive heart failure An increased risk of diabetes

VMA
Why Get Tested?

To help diagnose or rule out a neuroblastoma, pheochromocytoma, or other neuroendocrine tumor; to monitor the effectiveness of treatment
When to Get Tested?

If you are a child with an abdominal mass, bruising around the eyes, trouble walking, or bone pain; if you are someone with symptoms of increased catecholamine release, such as persistent or episodic high blood pressure, severe headaches, rapid heart rate, and sweating; when you have been treated for a neuroendocrine tumor, such as neuroblastoma or pheochromocytoma
Sample Required?

A 24-hour urine sample is preferred; sometimes a random urine sample is acceptable.

Test Preparation Needed?

This test is affected by caffeine, certain drugs, and stresses. Inform your doctor of any medications you are taking and follow any instructions you are given for things to avoid before sample collection.
What is being tested?

This test measures the amount of vanillylmandelic acid (VMA) that is excreted into the urine, typically over a 24-hour period. VMA is one of the metabolites of the catecholamines epinephrine (adrenaline) and norepinephrine. Catecholamines are a group of similar hormones produced in the adrenal medulla, the central portion of the adrenal glands. Adrenal glands are small triangular organs located on top of each kidney. The primary catecholamines that they release are dopamine, epinephrine, and norepinephrine. These hormones are released into the bloodstream in response to physical or emotional stress. They help transmit nerve impulses in the brain, increase glucose and fatty acid release for energy, dilate bronchioles, and dilate the pupils. Norepinephrine also constricts blood vessels, increasing blood pressure, and epinephrine increases heart rate and metabolism. After completing their actions, the catecholamines are metabolized to inactive compounds. Dopamine becomes homovanillic acid (HVA), norepinephrine breaks down into normetanephrine and VMA, and epinephrine becomes metanephrine and VMA. Both the hormones and their metabolites are excreted in the urine. VMA is usually present in the urine in small fluctuating amounts that only increase appreciably during and shortly after the body is exposed to a stressor. Neuroblastomas, pheochromocytomas, and other neuroendocrine tumors, however, can produce large amounts of catecholamines, resulting in greatly increased concentrations of the hormones and their metabolites. The hormone releases can cause persistent hypertension, severe headaches, palpitations, sweating, nausea, anxiety, and tingling in the extremities. Neuroblastomas are rare cancers but, according to the American Cancer Society, they are the most common cancers affecting infants. About 650 new cases are diagnosed each year, primarily in babies one to two years old, with up to 90% of cases occurring in children under the age of five. Rarely, a baby may be born with a neuroblastoma. Arising from primitive nerve cells, the cancers can be found in the abdomen, adrenal glands, neck, chest, or pelvis. About two thirds of the time, the tumors will have metastasized and moved to other parts of the body by the time they are diagnosed. Once detected, many neuroblastomas can be successfully treated. In some cases, neuroblastomas may disappear on their own or their cells may spontaneously mature into normal nerves cells, turning the neuroblastoma into a benign ganglioneuroma or into a partially benign ganglioneuroblastoma.

Pheochromocytomas are rare tumors that arise primarily in the adrenal glands. According to the National Cancer Institute, about 800 cases a year are diagnosed in the US, affecting primarily those in their thirties to fifties. Most pheochromocytomas are benign and do not spread from their original location, but some may metastasize and move to other organs within the body. Left untreated, they continue to grow and produce catecholamines, and hormone-related symptoms continue to worsen. Over time, the hypertension that the pheochromocytoma causes may damage body organs, such as the kidneys and heart, and raise the risk of an affected person having a stroke or heart attack. Pheochromocytomas are important to diagnose because they represent a potentially curable form of hypertension. In most cases, they can be removed and/or treated to reduce or eliminate the associated symptoms and complications. The VMA test, along with other tests for catecholamines and their metabolites, can be used to help detect the presence of neuroblastomas and pheochromocytomas.
How is the sample collected for testing?

For a 24-hour urine collection, all of your urine should be saved for a 24-hour period. It is important that the sample be refrigerated during this time period. A single urine sample is collected for a random urine test. NOTE: If undergoing medical tests makes you or someone you care for anxious, embarrassed, or even difficult to manage, you might consider reading one or more of the following articles: Coping with Test Pain, Discomfort, and Anxiety, Tips on Blood Testing, Tips to Help Children through Their Medical Tests, and Tips to Help the Elderly through Their Medical Tests.
Is any test preparation needed to ensure the quality of the sample?

This test is affected by caffeine, certain drugs, and stresses. Inform your doctor of any medications you are taking, including herbal supplements and over-the-counter products, and follow any instructions you are given for things to avoid before sample collection.
How is it used?

The VMA test is primarily used to detect and rule out neuroblastomas in children with an abdominal mass or other symptoms suggestive of the disease. It may be ordered along with an HVA test to help diagnose a neuroblastoma, to monitor the effectiveness of treatment, and to monitor for neuroblastoma recurrence. A urine creatinine is typically also ordered, especially with random urine testing, and tests results are frequently reported as VMA-to-creatinine and HVA-to-creatinine ratios. Since creatinine is a substance that is excreted in the urine at a relatively constant rate, it serves as a comparison to the rate of VMA and HVA excretion. A VMA test was once frequently ordered to detect pheochromocytomas, but the preferred tests are now plasma free metanephrines, urine metanephrines, and urine or plasma catecholamine tests. The VMA test may still be ordered along with one or more of these tests to help detect and rule out a pheochromocytoma.
When is it ordered?

VMA testing is ordered when a doctor either suspects that a child has a neuroblastoma or wants to rule out the possibility. She may order it, along with an HVA, when a child has an abdominal mass or other symptoms suggestive of a neuroblastoma. These signs and symptoms can vary depending upon their location and may vary over time:

Most neuroblastomas are located in the belly, so the most common symptom is an unusual non-tender lump or mass in that area. Others include abdominal pain and lack of appetite. Sometimes a tumor in the neck can cause swelling and affect swallowing or breathing. If the tumor presses on nerves by the spine, it may affect the child's ability to walk.

If it has spread to the bones (metastasized), it may cause bone pain, and if to the marrow, it may affect blood cell production, causing fatigue, pallor, and bleeding.

A neuroblastoma can also cause bruising around the eyes and drooping eyelids, bluish purple spots on the skin, and can sometimes affect bladder and bowel function. Rarely, it may cause muscle spasms and rapid eye movements referred to as "dancing eyes, dancing feet." The VMA test may also be ordered when a child has been treated for a neuroblastoma and ordered periodically to monitor for recurrence. A VMA test may be ordered along with one or more metanephrine or catecholamine tests when a doctor suspects that a person has a pheochromocytoma. They may be ordered when a person has persistent or recurring hypertension that is not responding to conventional therapies, to monitor the effectiveness of treatment for a pheochromocytoma, and periodically to monitor for recurrence.
What does the test result mean?

Since the VMA test is sensitive to many outside influences, and neuroblastomas and pheochromocytomas are rare, a doctor may see more false positives with this test than true positives. When a person has large amounts of VMA in his urine, it is not diagnostic of a condition; it is an indication that further investigation is necessary. Intense emotional and physical stresses can cause moderate to large temporary increases in VMA levels. The doctor must evaluate the child or adult as a whole his physical condition, emotional state, prescribed medications, and any over-the-counter products that he is taking. When interfering substances and/or conditions are found and resolved, the doctor will frequently re-test the person to determine whether the VMA is still elevated. The doctor may also order other catecholamine and metanephrine testing and imaging tests, such as an MRI, to help find the tumor(s). If a child or adult is being treated for a neuroblastoma or pheochromocytoma, then decreasing VMA concentrations indicate a response to treatment; stable or increasing levels indicate that the treatment is not effective. If a VMA concentration is initially normal and then increases in a person who has had a previous neuroblastoma or pheochromocytoma, then it is likely that the tumor is recurring. About 90% of children with a neuroblastoma will produce excess VMA and HVA. If a VMA and other catecholamine and metanephrine testing are normal, then it is less likely that a child has a neuroblastoma or an adult has a pheochromocytoma, but it cannot be ruled out. The tumors do not necessarily produce catecholamines at a constant rate, so increases in the hormones and metabolites will fluctuate in the urine and may not be increased in the sample tested. This is especially true when a single random urine sample is tested.
Is there anything else I should know?

The amount of VMA produced does not necessarily correspond to the size of the tumor. This is a physical characteristic of the tumor tissue. The total amount of VMA produced will tend to increase, however, as the tumor increases in size. A doctor will sometimes evaluate a VMA-to-HVA ratio with a neuroblastoma. A low ratio is associated with a poorer prognosis. A variety of medications can interfere with VMA testing, but those being tested should always consult with their doctor before discontinuing any prescribed medications. Some of the drugs that can interfere include appetite suppressants, caffeine, clonidine, disulfiram, histamine, imipramine, insulin, epinephrine, levodopa, lithium, morphine, MAO inhibitors, nitroglycerin, and rauwolfia alkaloids. The effects of these drugs on VMA results will be different from person to person and are often not predictable.

DD Racoon Eyes Racoon Eyes Raccoon eyes ato mata rakun, adalah ekimosis bilateral di daerah periorbital yang timbul tidak akibat dari trauma jaringan lunak muka. Biasanya raccoon eyes ini merupakan indikator dari fraktur basis cranii, yang terjadi ketika fraktur mengenai meningens dan mengakibatkan sinus-sinus vena berdarah ke vili arakhnoid. Racoon eyes ini bisa jadi merupakan satu-satunya tanda dari fraktur tulang tengkorak yang tidak terlihat di foto roentgen tengkorak. Jika ditemukan tanda ini, butuh pendekatan klinis secepatnya sebab fraktur basis cranii dapat mengakibatkan cedera nervus cranial, pembuluh darah dan batang otak. Setelah diketahui adanya raccoon eyes ini, lakukan pemeriksaan terhadap tanda vital pasien dan cari tahu kapan dan bagaimana bisa terjadi trauma kepala ini. Juga lakukan pemeriksaan terhadap trauma lainnya. Pemeriksaan dimulai dengan memeriksa tingkat kesadaran penderita menggunakan Glasgow Coma Scale (GCS). Berikutnya lakukan evaluasi terhadap fungsi dari saraf-saraf kranial, terutama nervus I (olfactory), III (occulomotoric), IV (trochlear), VI (abducens), dan VII (facial). Jika kondisi pasien memungkinkan, lakukan tes penglihatan dan tes dengar. Temukan dan catat semua kelainan di tulang muka, seperti pembengkakan, nyeri terlokalisir, Battle's sign, dan laserasi. Periksa juga adanya ekimosis pada daerah mastoid. Juga cari apakah ada perdarahan ataupun keluarnya cairan serebrospinal (LCS) dari hidung ataupun telinga. PENYEBAB Trauma pada basis kranii (basilar skull fracture) akan mengakibatkan raccoon eyes ini. Gejala yang dapat menyertainya berupa perdarahan dari faring, epistaksis, rhinnorea, otorrhea, dan bulging pada membran timpani akibat LCS ataupun darah. Pasien mungkin akan mengalami kesulitan mendengar, sakit kepala, nausea, muntah, palsy nervus cranial, dan gangguan kesadaran. Juga bisa didapatkan tanda Battle's yang positif. Penyebab lainnya dapat berupa raccoon eyes yang terjadi akibat pembedahan (post craniotomy), jika terjadi robekan pada meningens dan perdarahan kedalam sinus). LEBIH LANJUTNYA Pastikan pasien dalam bed rest total. Selanjutnya lakukan pemeriksaan tingkat kesadaran secara berkala, dan periksa tanda vitalnya setiap jam. Waspadalah jika terjadi bradipnu, bradikardi, hipertensi dan demam. Untuk menghindari lebih banyak robekan dan infeksi, jangan lakukan suction atau memasukkan nasogastic tube melalui hidung pasien. Lakukan pemantauan terhadap tandatanda dari meningitis seperti demam, dan kaku kuduk, juga dapat dilakukan pemberian terapi antibiotik profilaksis. Juga lakukan pemeriksaan lebih lanjut seperti radigrafi kepala, dan jika memungkinkan CTScan. Jika robekan pada dura tidak menghilang dengan sendirinya, lakukan pemeriksaan dengan

sisternografi untuk dapat menentukan dimana lokasi robekan, dan nantinya akan dilakukan operasi konstruktif.

differential diagnosis: The correct diagnosis of this condition is sometimes delayed because of workup for child abuse or trauma. There are a multitude of differential diagnoses for the presentation of periorbital edema and ecchymosis, e.g., 1. child abuse or trauma, 2. infection of the soft tissues associated with a spreading dental infection and an allergic reaction. 3. Other systemic causes to be considered include myxoedema, 4. other neoplasias such as lymphoma, amyloidosis, Kaposi's sarcoma, multiple myeloma, and neuroblastoma or 5. haematological coagulopathies such as haemophilia. note:

Neuroblastoma has been called the great mimicker because of its myriad clinical presentations related to the site of the primary tumor, metastatic disease, and its metabolic tumor by-products. Pepper syndrome occurs in infants with overwhelming metastatic neuroblastoma of the liver that results in respiratory compromise. Pepper syndrome was identified as a localized primary tumor and metastatic disease limited to the skin, liver, and bone marrow in infants. Pepper syndrome has since been associated with stage 4S neuroblastoma, a unique entity that occurs only in infants younger than 1 year. Pepper syndrome generally confers a better prognosis, as it is associated with spontaneous regression. "Blueberry muffin" babies are infants in whom neuroblastoma has metastasized to random subcutaneous sites. When provoked, the nodules become intensely red and subsequently blanch for several minutes thereafter. The response is probably secondary to the release of vasoconstrictive metabolic tumor by-products. These nodules can be diagnostic of neuroblastoma, but leukemic infiltrates that metastasize to the skin should be considered in the differential diagnoses when these children are evaluated. Widespread metastasis of neuroblastoma to the bone may result in Hutchinson syndrome, which results in bone pain with consequent limping and pathologic fractures. Neuroblastomas that arise in the paraspinal ganglia may invade through the neural foramina, compress the spinal cord, and subsequently cause paralysis

The most common finding upon physical examination is a nontender, firm, irregular abdominal mass that crosses the midline. In contrast, children who present with Wilms tumor have a smooth mobile flank mass that typically does not cross the midline.