A
pheochromocytoma or
phaeochromocytoma (
PCC) is a neuroendocrine tumor of the
medullaThe adrenal medulla is part of the adrenal gland. It is located at the center of the gland, being surrounded by the adrenal cortex. It is the innermost part of the adrenal gland, consisting of cells that secrete epinephrine , norepinephrine , and a small amount of dopamine in response to...
of the
adrenal glandIn mammals, the adrenal glands are endocrine glands that sit atop the kidneys; in humans, the right suprarenal gland is triangular shaped, while the left suprarenal gland is semilunar shaped...
s (originating in the
chromaffin cellChromaffin cells are neuroendocrine cells found in the medulla of the adrenal gland and in other ganglia of the sympathetic nervous system. They are modified post-synaptic sympathetic neurons that receive sympathetic input...
s), or extra-adrenal chromaffin tissue that failed to involute after birth and secretes excessive amounts of
catecholamineCatecholamines are molecules that have a catechol nucleus consisting of benzene with two hydroxyl side groups and a side-chain amine. They include dopamine, as well as the "fight-or-flight" hormones adrenaline and noradrenaline released by the adrenal medulla of the adrenal glands in response to...
s, usually
noradrenalineNorepinephrine is the US name for noradrenaline , a catecholamine with multiple roles including as a hormone and a neurotransmitter...
(
norepinephrineNorepinephrine is the US name for noradrenaline , a catecholamine with multiple roles including as a hormone and a neurotransmitter...
), and
adrenalineEpinephrine is a hormone and a neurotransmitter. It increases heart rate, constricts blood vessels, dilates air passages and participates in the fight-or-flight response of the sympathetic nervous system. In chemical terms, adrenaline is one of a group of monoamines called the catecholamines...
(
epinephrineEpinephrine is a hormone and a neurotransmitter. It increases heart rate, constricts blood vessels, dilates air passages and participates in the fight-or-flight response of the sympathetic nervous system. In chemical terms, adrenaline is one of a group of monoamines called the catecholamines...
) to a lesser extent. Extra-adrenal paragangliomas (often described as extra-adrenal pheochromocytomas) are closely related, though less common, tumors that originate in the
gangliaIn anatomy, a ganglion is a biological tissue mass, most commonly a mass of nerve cell bodies. Cells found in a ganglion are called ganglion cells, though this term is also sometimes used to refer specifically to retinal ganglion cells....
of the
sympathetic nervous systemThe sympathetic nervous system is one of the three parts of the autonomic nervous system, along with the enteric and parasympathetic systems. Its general action is to mobilize the body's nervous system fight-or-flight response...
and are named based upon the primary anatomical site of origin.
Signs and symptoms
The
signsSigns is the plural of sign. See sign .Signs may also refer to:*Signs , a 2002 film by M. Night Shyamalan*Signs , a journal of women's studies...
and symptoms of a pheochromocytoma are those of
sympathetic nervous systemThe sympathetic nervous system is one of the three parts of the autonomic nervous system, along with the enteric and parasympathetic systems. Its general action is to mobilize the body's nervous system fight-or-flight response...
hyperactivity, including:
- Skin sensations
- Flank
Flank may refer to:* Flank, the side of either a horse or a military unit*Flanking maneuver in military tactics* Flank , the waveform edge formed when a signal goes high or low...
pain
- Elevated heart rate
- Elevated blood pressure, including paroxysmal (sporadic, episodic) high blood pressure, which sometimes can be more difficult to detect; another clue to the presence of pheochromocytoma is orthostatic hypotension
Orthostatic hypotension, also known as postural hypotension, orthostasis, and colloquially as head rush or dizzy spell, is a form of hypotension in which a person's blood pressure suddenly falls when the person stands up or stretches. The decrease is typically greater than 20/10 mm Hg, and may be...
(a fall in systolic blood pressure greater than 20 mmHg or a fall in diastolic blood pressure greater than 10 mmHg upon standing)
- Palpitations
- Anxiety
Anxiety is a psychological and physiological state characterized by somatic, emotional, cognitive, and behavioral components. The root meaning of the word anxiety is 'to vex or trouble'; in either presence or absence of psychological stress, anxiety can create feelings of fear, worry, uneasiness,...
often resembling that of a panic attackPanic attacks are periods of intense fear or apprehension that are of sudden onset and of relatively brief duration. Panic attacks usually begin abruptly, reach a peak within 10 minutes, and subside over the next several hours...
- Diaphoresis
Diaphoresis is excessive sweating commonly associated with shock and other medical emergency conditions.Diaphoretic is the state of perspiring profusely, or something that has the power to cause increased perspiration....
(excessive sweating)
- Headaches
- Pallor
Pallor is a reduced amount of oxyhaemoglobin in skin or mucous membrane, a pale color which can be caused by illness, emotional shock or stress, stimulant use, lack of exposure to sunlight, anaemia or genetics....
- Weight loss
Weight loss, in the context of medicine, health or physical fitness, is a reduction of the total body mass, due to a mean loss of fluid, body fat or adipose tissue and/or lean mass, namely bone mineral deposits, muscle, tendon and other connective tissue...
- Localized amyloid
Amyloids are insoluble fibrous protein aggregates sharing specific structural traits. Abnormal accumulation of amyloid in organs may lead to amyloidosis, and may play a role in various neurodegenerative diseases.-Definition:...
deposits found microscopically
- Elevated blood glucose level (due primarily to catecholamine stimulation of lipolysis
Lipolysis is the breakdown of lipids and involves the hydrolysis of triglycerides into free fatty acids followed by further degradation into acetyl units by beta oxidation. The process produces Ketones, which are found in large quantities in ketosis, a metabolic state that occurs when the liver...
(breakdown of stored fat) leading to high levels of free fatty acids and the subsequent inhibition of glucose uptake by muscle cells. Further, stimulation of beta-adrenergic receptors leads to glycogenolysis and gluconeogenesis and thus elevation of blood glucose levels).
A pheochromocytoma can also cause resistant arterial hypertension. A pheochromocytoma can be fatal if it causes
malignant hypertensionMalignant hypertension or hypertensive emergency is severe hypertension with acute impairment of an organ system and the possibility of irreversible organ-damage...
, or severely high blood pressure. This hypertension is not well controlled with standard blood pressure medications.
Not all patients experience all of the signs and symptoms listed. The most common presentation is headache, excessive sweating, and increased heart rate, with the attack subsiding in less than one hour.
Tumors may grow very large, but most are smaller than 10 cm.
Statistics
- About 10% of adrenal cases are bilateral (suggesting hereditary disease)
- About 10% of adrenal cases occur in children (also suggesting hereditary disease)
- About 15% are extra-adrenal (located in any orthosympathetic tissue): of these 9% are in the abdomen and 1% are located elsewhere. Some extra-adrenal pheochromocytomas are probably actually paraganglioma
A paraganglioma is a rare neuroendocrine neoplasm that may develop at various body sites . About 97% are benign and cured by surgical removal; the remaining 3% are malignant because they are able to produce distant metastases...
s, but the distinction is only possible after surgical resection.
- About 11.1% of adrenal cases are malignant, but this rises to 30% for extra-adrenal cases
- About 26% are hereditary (earlier opinion had 10%)
- About 3% recur after being resected
- About 14% of affected individuals do not have arterial hypertension
Hypertension or high blood pressure is a cardiac chronic medical condition in which the systemic arterial blood pressure is elevated. What that means is that the heart is having to work harder than it should to pump the blood around the body. Blood pressure involves two measurements, systolic and...
(Campbell's Urology)
Cause
Up to 25% of pheochromocytomas may be familial. Mutations of the genes
VHL,
RET,
NF1NF1 can refer to:* Neurofibromatosis type I, a genetic disorder* Neurofibromin 1, a protein associated with the disorder above* Nuclear factor 1, a transcription factor...
(Gene 17 Neurofibromatosis type 1),
SDHBSuccinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial also known as iron-sulfur subunit of complex II is a protein that in humans is encoded by the SDHB gene....
and
SDHDSuccinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial , also known as succinate dehydrogenase complex subunit D , is a protein that in humans is encoded by the SDHD gene....
are all known to cause familial pheochromocytoma/extra-adrenal paraganglioma.
Pheochromocytoma is a
tumorA tumor or tumour is commonly used as a synonym for a neoplasm that appears enlarged in size. Tumor is not synonymous with cancer...
of the
multiple endocrine neoplasiaThe term multiple endocrine neoplasia encompasses several distinct syndromes featuring tumors of endocrine glands, each with its own characteristic pattern. In some cases, the tumors are malignant, in others, benign...
syndrome, type IIA and type IIB (also known as MEN IIA and MEN IIB, respectively). The other component neoplasms of that syndrome include parathyroid adenomas, and
medullary thyroid cancerMedullary thyroid cancer is a form of thyroid carcinoma which originates from the parafollicular cells , which produce the hormone calcitonin....
. Mutations in the autosomal
RET- People :* Ret Chol , Southern Sudanese politician from Nasir County of the Upper Nile state- Engineering and computer science :* Remote Electrical Tilt * Renewable energy target* Renewable energy Technology...
proto-oncogene drives these malignancies
.
Common mutations in the RET oncogene may also account for
medullary sponge kidneyMedullary sponge kidney is a congenital disorder of the kidneys characterized by cystic dilatation of the collecting tubules in one or both kidneys. In people with nephrolithiasis, the frequency of medullary sponge kidney has been reported by various authors to be 1221%. The disease is bilateral...
as well.
Pheochromocytoma linked to MEN II can be caused by RET oncogene mutations. Both syndromes are characterized by pheochromocytoma as well as
thyroid cancerThyroid neoplasm is a neoplasm or tumor of the thyroid. It can be a benign tumor such as thyroid adenoma, or it can be a malignant neoplasm , such as papillary, follicular, medullary or anaplastic thyroid cancer. Most patients are 25 to 65 years of age when first diagnosed; women are more affected...
(thyroid medullary carcinoma). MEN IIA also presents with hyperparathyroidism, while MEN IIB also presents with mucosal neuroma. It is now postulated that Lincoln suffered from MEN IIB, rather than Marfan's syndrome as previously thought, though this is uncertain.
Pheochromocytoma is also associated with
neurofibromatosisNeurofibromatosis is a genetically-inherited disorder in which the nerve tissue grows tumors that may be benign or may cause serious damage by compressing nerves and other tissues...
.
Diagnosis
The diagnosis can be established by measuring
catecholamineCatecholamines are molecules that have a catechol nucleus consisting of benzene with two hydroxyl side groups and a side-chain amine. They include dopamine, as well as the "fight-or-flight" hormones adrenaline and noradrenaline released by the adrenal medulla of the adrenal glands in response to...
s and
metanephrineMetanephrine is a metabolite of epinephrine created by action of catechol-O-methyl transferase on epinephrine. An article in the Journal of the American Medical Association, 2002, indicated that the measurement of plasma free metanephrines is the best tool in the diagnosis of pheochromocytoma, an...
s in plasma (blood) 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
stimulantStimulants are psychoactive drugs which induce temporary improvements in either mental or physical function or both. Examples of these kinds of effects may include enhanced alertness, wakefulness, and locomotion, among others...
s,
methyldopaMethyldopa is an alpha-adrenergic agonist psychoactive drug used as a sympatholytic or antihypertensive. Its use is now mostly deprecated following the introduction of alternative safer classes of agents...
,
dopamineDopamine is a catecholamine neurotransmitter present in a wide variety of animals, including both vertebrates and invertebrates. In the brain, this substituted phenethylamine functions as a neurotransmitter, activating the five known types of dopamine receptors—D1, D2, D3, D4, and D5—and their...
agonistAn agonist is a chemical that binds to a receptor of a cell and triggers a response by that cell. Agonists often mimic the action of a naturally occurring substance...
s, or ganglion blocking
antihypertensiveThe antihypertensives are a class of drugs that are used to treat hypertension . Evidence suggests that reduction of the blood pressure by 5 mmHg can decrease the risk of stroke by 34%, of ischaemic heart disease by 21%, and reduce the likelihood of dementia, heart failure, and mortality from...
s. Various foodstuffs (e.g.
vanillaVanilla is a flavoring derived from orchids of the genus Vanilla, primarily from the Mexican species, Flat-leaved Vanilla . The word vanilla derives from the Spanish word "", little pod...
ice creamIce cream is a frozen dessert usually made from dairy products, such as milk and cream, and often combined with fruits or other ingredients and flavours. Most varieties contain sugar, although some are made with other sweeteners...
) can also affect the levels of urinary
metanephrineMetanephrine is a metabolite of epinephrine created by action of catechol-O-methyl transferase on epinephrine. An article in the Journal of the American Medical Association, 2002, indicated that the measurement of plasma free metanephrines is the best tool in the diagnosis of pheochromocytoma, an...
and VMA (vanillylmandelic acid). Imaging by
computed tomographyX-ray computed tomography or Computer tomography , is a medical imaging method employing tomography created by computer processing...
or a
T2thumb|right|T2 relaxation curveSpin–spin relaxation is the mechanism by which Mxy, the transverse component of the magnetization vector, exponentially decays towards its equilibrium value of zero, in nuclear magnetic resonance and magnetic resonance imaging...
weighted
MRIMagnetic resonance imaging , nuclear magnetic resonance imaging , or magnetic resonance tomography is a medical imaging technique used in radiology to visualize detailed internal structures...
of the
headIn anatomy, the head of an animal is the rostral part that usually comprises the brain, eyes, ears, nose and mouth . Some very simple animals may not have a head, but many bilaterally symmetric forms do....
,
neckThe neck is the part of the body, on many terrestrial or secondarily aquatic vertebrates, that distinguishes the head from the torso or trunk. The adjective signifying "of the neck" is cervical .-Boner anatomy: The cervical spine:The cervical portion of the human spine comprises seven boney...
, and
chestThe chest is a part of the anatomy of humans and various other animals. It is sometimes referred to as the thorax or the bosom.-Chest anatomy - Humans and other hominids:...
, and
abdomenIn vertebrates such as mammals the abdomen constitutes the part of the body between the thorax and pelvis. The region enclosed by the abdomen is termed the abdominal cavity...
can help localize the tumor. Tumors can also be located using an MIBG scan, which is
scintigraphyScintigraphy is a form of diagnostic test used in nuclear medicine, wherein radioisotopes are taken internally, and the emitted radiation is captured by external detectors to form two-dimensional images...
using
iodine-123Iodine-123 is a radioactive isotope of iodine used in nuclear medicine imaging, including single photon emission computed tomography . The isotope's half-life is 13.22 hours; the decay by electron capture to tellurium-123 emits gamma radiation with predominant energies of 159 keV and 127 keV...
-marked metaiodobenzylguanidine.
Pheochromocytomas occur most often during young-adult to mid-adult life.
These tumors can form a pattern with other endocrine gland cancers which is labeled
multiple endocrine neoplasiaThe term multiple endocrine neoplasia encompasses several distinct syndromes featuring tumors of endocrine glands, each with its own characteristic pattern. In some cases, the tumors are malignant, in others, benign...
(MEN). Pheochromocytoma may occur in patients with MEN 2 and MEN 3 (MEN 2B). Von Hippel Lindau patients may also develop these tumors.
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.
Testing
- Blood Test
A blood test is a laboratory analysis performed on a blood sample that is usually extracted from a vein in the arm using a needle, or via fingerprick....
s: analysis of free metanephrineMetanephrine is a metabolite of epinephrine created by action of catechol-O-methyl transferase on epinephrine. An article in the Journal of the American Medical Association, 2002, indicated that the measurement of plasma free metanephrines is the best tool in the diagnosis of pheochromocytoma, an...
in blood plasma. High levels are indicative of pheochromocytoma
- Urine Tests
A urinalysis , also known as Routine and Microscopy , is an array of tests performed on urine, and one of the most common methods of medical diagnosis...
: Although this test is slightly less effective than plasma testing it is still considered highly effective in diagnosis. Usually the metabolites of norepinephrineNorepinephrine is the US name for noradrenaline , a catecholamine with multiple roles including as a hormone and a neurotransmitter...
and epinephrineEpinephrine is a hormone and a neurotransmitter. It increases heart rate, constricts blood vessels, dilates air passages and participates in the fight-or-flight response of the sympathetic nervous system. In chemical terms, adrenaline is one of a group of monoamines called the catecholamines...
, vanillylmandelic acid (VMA) and homovanillic acidHomovanillic acid is a major catecholamine metabolite...
(HVA) are found in relatively small amounts in normal humans. The increased intermittent excretion of these metabolites is indicative of the disease, but does not completely rule out other diseases which may cause the same excretion values.
- Other Tests:
- One diagnostic test used in the past for a pheochromocytoma is to administer clonidine
Clonidine is a sympatholytic medication used to treat medical conditions, such as high blood pressure, some pain conditions, ADHD and anxiety/panic disorder...
, 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.
- Chromogranin A
Chromogranin A or parathyroid secretory protein 1 is a member of the granin family of neuroendocrine secretory proteins, i.e. it is located in secretory vesicles of neurons and endocrine cells...
is elevated in case of pheochromocytoma.
- Another test is for the clinician to press gently on the adrenal gland
In mammals, the adrenal glands are endocrine glands that sit atop the kidneys; in humans, the right suprarenal gland is triangular shaped, while the left suprarenal gland is semilunar shaped...
. 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.
- Warning: Testing via histamine
Histamine is an organic nitrogen compound involved in local immune responses as well as regulating physiological function in the gut and acting as a neurotransmitter. Histamine triggers the inflammatory response. As part of an immune response to foreign pathogens, histamine is produced by...
and tyramineTyramine is a naturally occurring monoamine compound and trace amine derived from the amino acid tyrosine. Tyramine acts as a catecholamine releasing agent...
is dangerous and should not be used.
Tumor location
In adults, approximately 80% of pheochromocytomas are unilateral and solitary, 10% are bilateral, and 10% are extra-adrenal. In children, a fourth of tumors are bilateral, and an additional fourth are extra-adrenal. Solitary lesions inexplicably favor the right side. Although pheochromocytomas may grow to large size (>3 kg), most weigh <100 g and are <10 cm in diameter. Pheochromocytomas are highly vascular.
The tumors are made up of large, polyhedral, pleomorphic chromaffin cells. Fewer than 10% of these are malignant. As with several other endocrine tumors, malignancy cannot be determined from the histologic appearance; tumors that contain large number of aneuploid or tetraploid cells, as determined by flow cytometry, are more likely to recur. Local invasion of surrounding tissues or distant metastases indicate malignancy.
Extra-adrenal Pheochromocytomas:
Extra-adrenal pheochromocytomas usually weigh 20 to 40 g and are <5 cm in diameter. Most are located within the abdomen in association with the celiac, superior mesenteric, inferior mesenteric ganglia and
Organ of ZuckerkandlThe Organ of Zuckerkandl is a chromaffin body derived from neural crest located at the bifurcation of the aorta or at the origin of the inferior mesenteric artery...
. Approximately 10% are in the thorax, 1% are within the urinary bladder, and less than 3% are in the neck, usually in association with the sympathetic ganglia or the extracranial branches of the ninth cranial nerves.
Differential diagnosis
The
differential diagnosesA differential diagnosis is a systematic diagnostic method used to identify the presence of an entity where multiple alternatives are possible , and may also refer to any of the included candidate alternatives A differential diagnosis (sometimes abbreviated DDx, ddx, DD, D/Dx, or ΔΔ) is a...
of pheochromocytoma include:
- Anxiety disorder
Anxiety disorder is a blanket term covering several different forms of abnormal and pathological fear and anxiety. Conditions now considered anxiety disorders only came under the aegis of psychiatry at the end of the 19th century. Gelder, Mayou & Geddes explains that anxiety disorders are...
s
- Paraganglioma
A paraganglioma is a rare neuroendocrine neoplasm that may develop at various body sites . About 97% are benign and cured by surgical removal; the remaining 3% are malignant because they are able to produce distant metastases...
s
- Essential hypertension
Essential hypertension is the form of hypertension that by definition, has no identifiable cause. It is the most common type of hypertension, affecting 95% of hypertensive patients, it tends to be familial and is likely to be the consequence of an interaction between environmental and genetic...
- Hyperthyroidism
Hyperthyroidism is the term for overactive tissue within the thyroid gland causing an overproduction of thyroid hormones . Hyperthyroidism is thus a cause of thyrotoxicosis, the clinical condition of increased thyroid hormones in the blood. Hyperthyroidism and thyrotoxicosis are not synonymous...
- Insulinoma
An insulinoma is a tumour of the pancreas that is derived from beta cells and secretes insulin.Beta cells secrete insulin in response to increases in blood glucose. The resulting increase in insulin acts to lower blood glucose back to normal levels at which point further secretion of insulin is...
- Mercury poisoning
Mercury poisoning is a disease caused by exposure to mercury or its compounds. Mercury is a heavy metal occurring in several forms, all of which can produce toxic effects in high enough doses...
- Paroxysmal supraventricular tachycardia
- Renovascular hypertension
Renovascular hypertension is a syndrome which consists of high blood pressure caused by narrowing of the arteries supplying the kidneys...
- Carcinoid
Carcinoid is a slow-growing type of neuroendocrine tumor, originating in the cells of the neuroendocrine system.In 2000, the World Health Organization redefined "carcinoid", but this new definition has not been accepted by all practitioners. This has led to some complexity in distinguishing...
Treatment
Surgical
resectionSegmental resection is a surgical procedure to remove part of an organ or gland. It may also be used to remove a tumor and normal tissue around it. In lung cancer surgery, segmental resection refers to removing a section of a lobe of the lung.- External links :* entry in the public domain NCI...
of the tumor is the treatment of first choice, either by open
laparotomyA laparotomy is a surgical procedure involving a large incision through the abdominal wall to gain access into the abdominal cavity. It is also known as coeliotomy.- Terminology :...
or else
laparoscopyLaparoscopy is an operation performed in the abdomen or pelvis through small incisions with the aid of a camera...
. Given the complexity of
perioperativeThe perioperative period, less commonly spelled the peroperative period, is the time period describing the duration of a patient's surgical procedure; this commonly includes ward admission, anesthesia, surgery, and recovery. Perioperative generally refers to the three phases of surgery:...
management, and the potential for catastrophic intra and postoperative complications, such surgery should be performed only at centers experienced in the management of this disorder. In addition to the surgical expertise that such centers can provide, they will also have the necessary endocrine and anesthesia resources. It may also be necessary to carry out adrenalectomy, a complete surgical removal of the affected adrenal gland(s).
Either surgical option requires prior treatment with the non-specific and irreversible alpha adrenoceptor blocker
PhenoxybenzaminePhenoxybenzamine is a non-selective, irreversible alpha antagonist.-Uses:It is used in the treatment of hypertension, and specifically that caused by pheochromocytoma...
(Irreversible blockade is important because a massive release of catecholamines from the tumor may overcome a reversible blockade). Doing so permits the surgery to proceed while minimizing the likelihood of severe intraoperative hypertension (as might occur when the tumor is manipulated). Some authorities would recommend that a combined alpha/beta blocker such as
labetalolLabetalol is a mixed alpha/beta adrenergic antagonist, which is used to treat high blood pressure.-Indications:...
also be given in order to slow the heart rate. Regardless, a "pure" beta blocker such as
atenolol must never be used in the presence of a pheochromocytoma due to the risk of such treatment's leading to unopposed alpha agonism and, thus, severe and potentially refractory hypertension.
The patient with pheochromocytoma is invariably volume depleted. In other words, the chronically elevated
adrenergicAn adrenergic agent is a drug, or other substance, which has effects similar to, or the same as, epinephrine . Thus, it is a kind of sympathomimetic agent...
state characteristic of an untreated pheochromocytoma leads to near-total inhibition of
reninRenin , also known as an angiotensinogenase, is an enzyme that participates in the body's renin-angiotensin system -- also known as the Renin-Angiotensin-Aldosterone Axis -- that mediates extracellular volume , and arterial vasoconstriction...
-
angiotensinAngiotensin, a peptide hormone, causes blood vessels to constrict, and drives blood pressure up. It is part of the renin-angiotensin system, which is a major target for drugs that lower blood pressure. Angiotensin also stimulates the release of aldosterone, another hormone, from the adrenal cortex...
activity, resulting in excessive fluid loss in the urine and thus reduced blood volume. Hence, once the pheochromocytoma has been resected, thereby removing the major source of circulating catecholamines, a situation arises where there is both very low sympathetic activity and volume depletion. This can result in profound
hypotensionIn physiology and medicine, hypotension is abnormally low blood pressure, especially in the arteries of the systemic circulation. It is best understood as a physiologic state, rather than a disease. It is often associated with shock, though not necessarily indicative of it. Hypotension is the...
. Therefore, it is usually advised to "salt load" pheochromocytoma patients before their surgery. This may consist of simple interventions such as consumption of high salt food pre-operatively, direct salt replacement or through the administration of intravenous saline solution.
Epidemiology
Pheochromocytoma is seen in between 2–8 in 1,000,000, with approximately 1000 cases diagnosed in United States yearly. It mostly occurs in young or middle age adults, though presents earlier in hereditary cases.
History
In 1886, Fränkel made the first description of a patient with pheochromocytoma; however, the term was first coined by
Ludwig PickProfessor Ludwig Pick was a German pathologist who was a native of Landsberg an der Warthe. In 1893 he earned his medical doctorate in Leipzig, and subsequently practiced medicine at Leopold Landau's private Frauenklinik, where he remained until 1906...
, a pathologist, in 1912. In 1926, Roux (in Switzerland) and Mayo (in U.S.A.) were the first surgeons to remove pheochromocytomas.
Cell lines
The PC12 cell line was derived from rat pheochromocytoma by Greene and Tischler in the 1970's.
Society and culture
- Pheochromocytomas were mentioned a few times in the NBC TV series ER
ER is an American medical drama television series created by novelist Michael Crichton that aired on NBC from September 19, 1994 to April 2, 2009. It was produced by Constant c Productions and Amblin Entertainment, in association with Warner Bros. Television...
(aired in 1994–2009) and usually generated interest from the doctors due to the rarity of the diagnosis.
- In the second season premiere of the TV series House
House is an American television medical drama that debuted on the Fox network on November 16, 2004. The show's central character is Dr. Gregory House , an unconventional and misanthropic medical genius who heads a team of diagnosticians at the fictional Princeton-Plainsboro Teaching Hospital in...
in the episode "Acceptance" (aired on 13 September 2005), House's patient, a death row inmate Clarence (played by LL Cool JJames Todd Smith , better known as LL Cool J , is an American rapper, entrepreneur, and actor...
), was diagnosed with pheochromocytoma — he killed his fourth victim unintentionally, during an episode of anger caused by adrenaline spike from the tumor.
- In Heaven Sword and Dragon Sabre (along with its various film, television and graphic novel adaptations), the third novel on the popular Condor Trilogy
The Condor Trilogy is a trilogy of three wuxia novels by Jin Yong.The novels in the trilogy are:*The Legend of the Condor Heroes *The Return of the Condor Heroes...
, the martial artist Xie Xun suffers from an 'endocrine disorder' that is most likely a pheochromocytoma. This disorder, caused by his mastery of Kongtong SectThe Kongtong Sect appears as one of the major orthodox Chinese martial arts sects in Jin Yong's wuxia novel The Heaven Sword and Dragon Saber. Its home base is in the Kongtong Mountains.-History:...
's Seven Harms Fist, causes him to suffer bouts of 'insanity' sporadically in a manner similar to that of HeraclesHeracles ,born Alcaeus or Alcides , was a divine hero in Greek mythology, the son of Zeus and Alcmene, foster son of Amphitryon and great-grandson of Perseus...
, flying into violent rages and wrongly identifying even his loved ones as his hated enemies.
- In the third season of the TV series Private Practice in the episode "The Way We Were" (aired on 8 October 2009), 11-year-old patient (Tammy Larsen, played by Emily Evan Rae
Emily Evan Rae Argenti is an American teen actress.-Filmography:- Awards/Nominations:-External links:...
) suffers from ectopic pheochromocytoma on her ovary, which caused her episodes of anger, and during one of them she stabbed her father with a knife.
- An episode of Mystery Diagnosis
Mystery Diagnosis is a television program that airs on the OWN: Oprah Winfrey Network. Each episode focuses on two or more individuals and their struggles to find out what ailments they suffer from....
dealt with a woman who had a pheochromocytoma.
- In season 10 of TV series MASH 4077 in the episode "Sons and Bowlers" Hawkeye receives news that his father is undergoing surgery after having been diagnosed with pheochromocytoma.
- British actress Katrin Cartlidge
Katrin Cartlidge was an English actress. She first appeared on screen as Lucy Collins in the Liverpool soap opera Brookside from 1982 to 1988 and later became well known for her film work with directors such as Mike Leigh and Lars von Trier.- Biography :Cartlidge was born in London to an English...
died at the age of 41 due to complications from Pheochromocytoma.
- President Eisenhower's death may have been caused by a pheochromocytoma.
- A brilliant skit during the Harvard Medical School annual show in 1978 included a song entitled "Pheo" (recited exactly in the manner of the Western song "Ringo") as a parody of a guest professor whose lecture on hypertension causes was oddly dominated by the extremely rare pheochromocytoma. The song began "The pulse I was feeling was thready and fast/So I thought I'd come upon at last/The cause of hypertension of Harvard fame/My steel-trap mind recalled the name of Pheo ...".
External links