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Encyclopedia > Pheochromocytoma
Pheochromocytoma
Classification & external resources
Medulla visible at bottom right.
ICD-10 C74.1
ICD-9 255.6
ICD-O: M8700/0
OMIM 171300
DiseasesDB 9912
eMedicine med/1816  radio/552 ped/1788
MeSH D010673

A phaeochromocytoma (pheochromocytoma in the US) is a neuroendocrine tumor of the medulla of the adrenal glands originating in the chromaffin cells, which secretes excessive amounts of catecholamines, usually adrenaline and noradrenaline (epinephrine and norepinephrine in the US). Extra-adrenal paragangliomas (often described as extra-adrenal pheochromocytomas) are closely related, though less common, tumors that originate in the ganglia of the sympathetic nervous system and are named based upon the primary anatomical site of origin. Image File history File links Gray1185. ... The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The International Statistical Classification of Diseases and Related Health Problems 10th Revision (ICD-10) is a coding of diseases and signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or diseases, as classified by the World Health Organization (WHO). ... // C00-D48 - Neoplasms (C00-C14) Malignant neoplasms, lip, oral cavity and pharynx (C00) Malignant neoplasm of lip (C01) Malignant neoplasm of base of tongue (C02) Malignant neoplasm of other and unspecified parts of tongue (C03) Malignant neoplasm of gum (C04) Malignant neoplasm of floor of mouth (C05) Malignant neoplasm of... The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. ... The International Classification of Diseases for Oncology (ICD-O) is a domain specific extension of the International Statistical Classification of Diseases and Related Health Problems for tumor diseases. ... The International Classification of Diseases for Oncology (ICD-O) is a domain specific extension of the International Statistical Classification of Diseases and Related Health Problems for tumor diseases. ... The Mendelian Inheritance in Man project is a database that catalogues all the known diseases with a genetic component, and - when possible - links them to the relevant genes in the human genome. ... The Disease Bold textDatabase is a free website that provides information about the relationships between medical conditions, symptoms, and medications. ... eMedicine is an online clinical medical knowledge base that was founded in 1996. ... Medical Subject Headings (MeSH) is a huge controlled vocabulary (or metadata system) for the purpose of indexing journal articles and books in the life sciences. ... Neuroendocrine tumors, or more properly gastro-entero-pancreatic or gastroenteropancreatic neuroendocrine tumors (GEP-NETs), are cancers of the interface between the endocrine (hormonal) system and the nervous system. ... In mammals, the adrenal glands are the triangle-shaped endocrine glands that sit atop the kidneys. ... In mammals, the adrenal glands (also known as suprarenal glands) are the triangle-shaped endocrine glands that sit on top of the kidneys; their name indicates that position (ad-, near or at + -renes, kidneys). They are chiefly responsible for regulating the stress response through the synthesis of corticosteroids and catecholamines... Chromaffin cells are neuroendocrine cells found in the medulla of the adrenal gland and in other ganglia of the sympathetic nervous system. ... tyrosine is the precursor of catecholamines epinephrine norepinephrine dopamine Synthesis Catecholamines are chemical compounds derived from the amino acid tyrosine containing catechol and amine groups. ... Epinephrine (INN) or adrenaline (BAN) is a hormone and a neurotransmitter. ... Norepinephrine, known as noradrenaline outside the USA, is a catecholamine and a phenethylamine with chemical formula C8H11NO3. ... A paraganglioma is a rare neoplasm that can be found in the head and neck region and other less common areas. ... This is a dorsal root ganglion (DRG) from a chicken embryo (around stage of day 7) after incubation overnight in NGF growth medium stained with anti-neurofilament antibody. ... Grays FIG. 838– The right sympathetic chain and its connections with the thoracic, abdominal, and pelvic plexuses. ...


Traditionally it is known as the "10% tumor":

  • bilateral disease is present in approximately 10% of patients
  • approximately 10% of tumours are malignant
  • approximately 10% are located in chromaffin tissue outside of the adrenal gland

Contents

Inheritance

Up to 25% of pheochromocytomas may be familial. Mutations of the genes VHL, RET, NF1, SDHB and SDHD are all known to cause familial pheochromocytoma/extra-adrenal paraganglioma. Von Hippel-Lindau disease (VHL) is a rare inherited genetic condition involving the abnormal growth of tumors in parts of the body which are particularly rich in blood supply. ... SDHB stands for succinate dehydrogenase complex subunit B. It is involved in the oxidation of succinate (succinate + ubiquinone = fumarate + ubiquinol) and carries electrons from FADH to CoQ. It is composed of four nuclear-encoded subunits. ... SDHD, which stands for succinate dehydrogenase complex subunit D, is one of the two transmembrane subunits of the four-subunit succinate dehydrogenase protein complex of the mitochondrial-respiratory chain (electron transfer chain), where it is described as complex II, and as it also participates in the citric acid cycle, in...


Features

The signs and symptoms of a pheochromocytoma are those of sympathetic nervous system hyperactivity, including: Look up signs in Wiktionary, the free dictionary. ... The term symptom (from the Greek syn = con/plus and pipto = fall, together meaning co-exist) has two similar meanings in the context of physical and mental health: A symptom can be a physical condition which shows that one has a particular illness or disorder (see e. ... Grays FIG. 838– The right sympathetic chain and its connections with the thoracic, abdominal, and pelvic plexuses. ... Hyperactivity can be described as a state in which a person is abnormally easily excitable and exuberant. ...

A pheochromocytoma can also cause resistant arterial hypertension. A pheochromocytoma can be fatal if it causes malignant hypertension, or severely high blood pressure. Tachycardia is an abnormally rapid beating of the heart, defined as a resting heart rate of over 100 beats per minute. ... For other forms of hypertension, see Hypertension (disambiguation). ... Orthostatic hypotension (also known as postural hypotension and, colloquially, as head rush or a dizzy spell) is a sudden fall in blood pressure, typically greater than 20/10 mm Hg, that occurs when a person assumes a standing position. ... Blood pressure is the pressure exerted by the blood on the walls of the blood vessels. ... One way of defining pressure is in terms of the height of a column of fluid that may be supported by that pressure; or the height of a column of fluid that exerts that pressure at its base. ... A palpitation is an abnormal, rapid beating of the heart, brought on by overexertion, disease or drugs. ... This article includes a list of works cited or a list of external links, but its sources remain unclear because it lacks in-text citations. ... It has been suggested that this article or section be merged into Panic Disorder. ... Diaphoresis is excessive sweating commonly associated with shock and other medical emergency conditions. ... A headache is a condition of mild to severe pain in the head; sometimes upper back or neck pain may also be interpreted as a headache. ... This article does not cite any references or sources. ... This article needs additional references or sources for verification. ... Arterial hypertension, or high blood pressure is a medical condition where the blood pressure is chronically elevated. ... Malignant hypertension is a complication of hypertension characterized by very elevated blood pressure, and organ damage in the eyes, brain, lung and/or kidneys. ... Arterial hypertension, or high blood pressure is a medical condition where the blood pressure is chronically elevated. ...


Not all patients experience all of the signs and symptoms listed.


Diagnosis

Histopathology of adrenal pheochromocytoma. Adrenectomy specimen.
Histopathology of adrenal pheochromocytoma. Adrenectomy specimen.

The diagnosis can be established by measuring catecholamines and metanephrines in plasma or through a 24-hour urine collection. Care should be taken to rule out other causes of adrenergic (adrenalin-like) excess like hypoglycemia, stress, exercise, and drugs affecting the catecholamines like methyldopa, dopamine agonists, or ganglion blocking antihypertensives. Various foodstuffs (e.g. vanilla ice cream) can also affect the levels of urinary metanephrine and VMA (vanillyl mandelic acid). Imaging by computed tomography or a T2 weighted MRI of the head, neck, and chest, and abdomen can help localize the tumor. Tumors can also be located using Iodine-131 meta-iodobenzylguanidine (I131 MIBG) imaging. Image File history File links Metadata No higher resolution available. ... Image File history File links Metadata No higher resolution available. ... Image File history File links Adrenaline. ... Image File history File links Adrenaline. ... Adrenaline redirects here. ... Chemical structure of norepinephrine (generated at Wikisophia. ... Chemical structure of norepinephrine (generated at Wikisophia. ... Norepinephrine (INN)(abbr. ... Catecholamines are chemical compounds derived from the amino acid tyrosine that act as hormones or neurotransmitters. ... Methyldopa or alpha-methyldopa (brand names Aldomet, Apo-Methyldopa, Dopamet, Novomedopa) is a centrally-acting antiadrenergic antihypertensive medication. ... Dopamine is a phenethylamine naturally produced by the human body. ... Agonists An agonist is a substance that binds to a receptor and triggers a response in the cell. ... Antihypertensives are a class of drugs that are used in medicine and pharmacology to treat hypertension (high blood pressure). ... Vanilla pods Vanilla is a flavouring derived from orchids in the genus Vanilla native to Mexico. ... Missing image Ice cream is often served on a stick Boxes of ice cream are often found in stores in a display freezer. ... A metabolite of epinephrine (created by action of catechol O-methyltransferase on epinephrine). ... Vanillyl mandelic acid (VMA) is a chemical end product of catecholamine metabolism. ... This article does not cite any references or sources. ... Magnetic Resonance Image showing a median sagittal cross section through a human head. ... “Human Head” redirects here. ... A human neck. ... Male Chest The chest is a part of the anatomy of humans and various other animals. ... The abdomen is a part of the body. ... Iodine-131 (131I), also called radioiodine, is a radioisotope of iodine. ...


One diagnostic test used in the past for a pheochromocytoma is to administer clonidine, a centrally-acting alpha-2 agonist used to treat high blood pressure. Clonidine mimics catecholamines in the brain, causing it to reduce the activity of the sympathetic nerves controlling the adrenal medulla. A healthy adrenal medulla will respond to the Clonidine suppression test by reducing catecholamine production; the lack of a response is evidence of pheochromocytoma. Clonidine is a centrally acting antihypertensive (to lower high blood pressure) agent, used mainly for this purpose in the past. ... Clonidine is a centrally acting antihypertensive (to lower high blood pressure) agent, used mainly for this purpose in the past. ...


Another test is for the clinician to press gently on the adrenal gland. A pheochromocytoma will often release a burst of catecholamines, with the associated signs and symptoms quickly following. This method is not recommended because of possible complications arising from a potentially massive release of catecholamines. In mammals, the adrenal glands (also known as suprarenal glands) are the triangle-shaped endocrine glands that sit on top of the kidneys; their name indicates that position (ad-, near or at + -renes, kidneys). They are chiefly responsible for regulating the stress response through the synthesis of corticosteroids and catecholamines...


Pheochromocytomas occur most often during young-adult to mid-adult life. Less than 10% of pheochromocytomas are malignant (cancerous), bilateral or pediatric. In medicine, malignant is a clinical term that means to be severe and become progressively worse, as in malignant hypertension. ...


These tumors can form a pattern with other endocrine gland cancers which is labeled multiple endocrine neoplasia (MEN). Pheochromocytoma may occur in patients with MEN 2 and MEN 3. VHL (Von Hippel Lindau) patients may also develop these tumors. Multiple endocrine neoplasia (MEN) (or multiple endocrine adenomas, or multiple endocrine adenomatosis -- MEA) consists of three syndromes featuring tumors of endocrine glands, each with its own characteristic pattern. ... Von Hippel-Lindau disease (VHL) is a rare inherited genetic condition involving the abnormal growth of tumors in parts of the body which are particularly rich in blood supply. ...


Patients experiencing symptoms associated with pheochromocytoma should be aware that it is rare. However, it often goes undiagnosed until autopsy; therefore patients might wisely choose to take steps to provide a physician with important clues, such as recording whether blood pressure changes significantly during episodes of apparent anxiety.


Differential diagnosis

The differential diagnosis of pheochromocytoma includes: This article or section does not cite any references or sources. ...

This article needs additional references or sources for verification. ... Carcinoid syndrome refers to the array of symptoms that occur secondary to carcinoid tumors. ... A paraganglioma is a rare neoplasm that can be found in the head and neck region and other less common areas. ... Essential hypertension is a subtype of arterial hypertension in which no one specific etiology can be isolated as the cause of increased blood pressure. ... Hyperthyroidism (or overactive thyroid gland) is the clinical syndrome caused by an excess of circulating free thyroxine (T4) or free triiodothyronine (T3), or both. ... An insulinoma is a tumour of the pancreas derived from the beta cells which while retaining the ability to synthesize and secrete insulin is autonomous of the normal feedback mechanisms. ... A supraventricular tachycardia (SVT) is a rapid rhythm of the heart in which the origin of the electrical signal is either the atria or the AV node. ... Renovascular hypertension (or renal hypertension) is a form of secondary hypertension. ...

Treatment

Surgical resection of the tumor is the treatment of first choice. Given the complexity of perioperative management, and the potential for catastrophic intra and postoperative complications, such surgery should be performed only at centers experienced in the area. In addition to the surgical expertise that such centers can provide, they will also have the necessary endocrine and anesthesia resources as well. It may also be nescessary to carry out adrenalectomy, a complete surgical removal of the affected adrenal gland(s). Resection is a method of orientation (direction or position finding) using a compass and topographic map. ... The perioperative period is the time period surrounding a patients surgical procedure; this commonly includes ward admission, anesthesia, surgery and recovery. ...


Either surgical option requires prior treatment with both the non-specific alpha adrenoceptor blocker Phenoxybenzamine to counteract hypertension and the beta-1 adrenoceptor antagonist Atenolol to reduce cardiac output. Given before surgery, these can also block the effect of a sudden release of adrenaline during tumour removal, which would otherwise endanger the anaethetised patient. Phenoxybenzamine is a non-specific, irreversible alpha blocker used in the treatment of hypertension, and specifically that caused by pheochromocytoma. ... Atenolol is a drug belonging to the group of beta blockers, a class of drugs used primarily in cardiovascular diseases. ...


External links


  Results from FactBites:
 
Pheochromocytoma - Wikipedia, the free encyclopedia (441 words)
A pheochromocytoma (also phaeochromocytoma, English spelling) is a tumor of the medulla of the adrenal glands originating in the chromaffin cells, which secretes excessive amounts of catecholamines, usually epinephrine and norepinephrine.
Extra-adrenal paragangliomas (often described as extra-adrenal pheochromocytomas) are closely related, though less common, tumors that originate in the ganglia of the sympathetic nervous system and are named based upon the primary anatomical site of origin.
One diagnostic test used in the past for a pheochromocytoma is to administer clonidine (Catapres®), a centrally-acting alpha-2 agonist used to treat high blood pressure.
Biochemical Diagnosis of Pheochromocytoma (5491 words)
Pheochromocytomas, differ from the adrenal medulla in that the tumors mainly secrete norepinephrine, whereas the predominant catecholamine secreted by the adrenal medulla is epinephrine.
In patients with pheochromocytoma, over 94% of the elevated plasma concentrations of normetanephrine or metanephrine are derived from metabolism of catecholamines by the catechol-O-methyltransferase within pheochromocytoma tumor cells and not by actions of extra-adrenal catechol-O-methyltransferase on catecholamines released by tumors into the circulation (41).
1995 Plasma metanephrines in the diagnosis of pheochromocytoma.
  More results at FactBites »

 
 

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