Pheochromocytoma differential diagnosis: Difference between revisions

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==Overview==
==Overview==
Pheochromocytoma must be differentiated from other causes of paroxysmal hypertension including: severe paroxysmal hypertension (Pseudopheochromocytoma)
Pheochromocytoma must be differentiated from other causes of paroxysmal hypertension including severe paroxysmal hypertension (Pseudopheochromocytoma)


, [[Panic disorder|panic disorder,]] [[Factitious hypertension]], [[carcinoid syndrome]], [[Migraine|Migraine headache]], [[Hyperthyroidism|Hyperthyroidism,]] [[Renovascular hypertension|Renovascular hypertension,]] [[Hypoglycemia]], Labile hypertension ([[White coat hypertension]]), [[Stroke|Stroke and compression of lateral medulla]], [[Seizure|Seizures]], [[Baroreflex failure]] and drugs.
, [[Panic disorder|panic disorder,]] [[Factitious hypertension]], [[carcinoid syndrome]], [[Migraine|Migraine headache]], [[Hyperthyroidism|Hyperthyroidism,]] [[Renovascular hypertension|Renovascular hypertension,]] [[Hypoglycemia]], Labile hypertension ([[White coat hypertension]]), [[Stroke|Stroke and compression of the lateral medulla]], [[Seizure|Seizures]], [[Baroreflex failure]] and drugs.


== Differentiating pheochromocytoma from other diseases ==
== Differentiating pheochromocytoma from other diseases ==
Pheochromocytoma must be differentiated from other causes of paroxysmal hypertension including: severe paroxysmal hypertension (Pseudopheochromocytoma)
Pheochromocytoma must be differentiated from other causes of paroxysmal hypertension including severe paroxysmal hypertension (Pseudopheochromocytoma), [[Panic disorder|panic disorder,]] [[Factitious hypertension]], [[carcinoid syndrome]], [[Migraine|Migraine headache]], [[Hyperthyroidism|Hyperthyroidism,]] [[Renovascular hypertension|Renovascular hypertension,]] [[Hypoglycemia]] and drugs.
 
, [[Panic disorder|panic disorder,]] [[Factitious hypertension]], [[carcinoid syndrome]], [[Migraine|Migraine headache]], [[Hyperthyroidism|Hyperthyroidism,]] [[Renovascular hypertension|Renovascular hypertension,]] [[Hypoglycemia]] and drugs.
{| class="wikitable"
{| class="wikitable"
!Disease
!Disease
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* [[Orthostatic hypotension]]
* [[Orthostatic hypotension]]
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|
'''High-risk patients''': plasma fractionated metanephrines is the first test if elevated; 24-hour urinary fractionated metanephrines, catecholamines, and imaging should be the second test for diagnosis. <sup>[[Pheochromocytoma laboratory findings#cite note-pmid11903030-2|[2]]]</sup>
'''High-risk patients''': plasma fractionated [[Metanephrine|metanephrines]] are the first test if elevated; 24-hour urinary fractionated metanephrines, catecholamines, and imaging should be the second test for diagnosis. <sup>[[Pheochromocytoma laboratory findings#cite note-pmid11903030-2|[2]]]</sup>


'''Low-risk patients''': 24-hour urinary fractionated catecholamines and metanephrines.<sup>[[Pheochromocytoma laboratory findings#cite note-pmid12574179-4|[4]]]</sup>
'''Low-risk patients''': 24-hour urinary fractionated [[catecholamines]] and [[Metanephrine|metanephrines]].<sup>[[Pheochromocytoma laboratory findings#cite note-pmid12574179-4|[4]]]</sup>
|-
|-
|Pseudopheochromocytoma
|Pseudopheochromocytoma
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Acute onset of high blood pressure, headache, chest pain, nausea, palpitations, flushing. Duration of attacks ranges from minutes to hours.
Acute onset of high blood pressure, headache, chest pain, nausea, palpitations, flushing. Duration of attacks ranges from minutes to hours.


|
|Increase in plasma catecholamines between and during attacks.<ref name="pmid8824124">{{cite journal| author=Mann SJ| title=Severe paroxysmal hypertension. An automatic syndrome and its relationship to repressed emotions. | journal=Psychosomatics | year= 1996 | volume= 37 | issue= 5 | pages= 444-50 | pmid=8824124 | doi=10.1016/S0033-3182(96)71532-3 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8824124  }}</ref><ref name="pmid17921824">{{cite journal| author=Sharabi Y, Goldstein DS, Bentho O, Saleem A, Pechnik S, Geraci MF et al.| title=Sympathoadrenal function in patients with paroxysmal hypertension: pseudopheochromocytoma. | journal=J Hypertens | year= 2007 | volume= 25 | issue= 11 | pages= 2286-95 | pmid=17921824 | doi=10.1097/HJH.0b013e3282ef5fac | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17921824  }}</ref>
|Increase in plasma catecholamines between and during attacks.<ref name="pmid8824124">{{cite journal| author=Mann SJ| title=Severe paroxysmal hypertension. An automatic syndrome and its relationship to repressed emotions. | journal=Psychosomatics | year= 1996 | volume= 37 | issue= 5 | pages= 444-50 | pmid=8824124 | doi=10.1016/S0033-3182(96)71532-3 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8824124  }}</ref><ref name="pmid17921824">{{cite journal| author=Sharabi Y, Goldstein DS, Bentho O, Saleem A, Pechnik S, Geraci MF et al.| title=Sympathoadrenal function in patients with paroxysmal hypertension: pseudopheochromocytoma. | journal=J Hypertens | year= 2007 | volume= 25 | issue= 11 | pages= 2286-95 | pmid=17921824 | doi=10.1097/HJH.0b013e3282ef5fac | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17921824  }}</ref>
|-
|-
|Panic attacks
|[[Panic attacks]]
|
|
* Paroxysms of increased sympathetic activity.
* Paroxysms of increased sympathetic activity.
* Characterized by episodes of fear or panic
* It is characterized by episodes of fear or panic
* Such as pseudo pheochromocytoma, panic disorders are usually  
* Such as [[Pseudopheochromocytoma|pseudo-pheochromocytoma]], panic disorders are usually associated with physical symptoms such as chest pain, headache,                palpitations, flushing and both respond to antidepressants.  
associated with physical symptoms such as chest pain, headache,                palpitations, flushing and both respond to antidepressants.  
|In panic attacks, fear precedes physical symptoms. By contrast, in pseudo pheochromocytoma physical symptoms occur before feeling fear.
 
''<u>In panic attacks, fear precedes physical symptoms. By contrast, in pseudo pheochromocytoma physical symptoms occur before feeling fear.</u>''
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|
|
Laboratory studies that can exclude medical disorders other than panic disorder include:
Laboratory studies that can exclude medical disorders other than panic disorder include:
* Serum electrolytes to
* Serum electrolytes  
* Serum glucose
* [[Serum glucose]]
* Cardiac enzymes
* [[Cardiac enzymes]]
* Urine toxicology screening.
* Urine toxicology screening
|-
|-
|Labile hypertension (White coat hypertension)
|Labile hypertension (White coat hypertension)
|Patients exhibit elevated [[blood pressure]] in a clinical setting but not in other settings.<sup>[[Chronic hypertension differential diagnosis#cite note-pmid24107724-1|[1]]]</sup>   
|Patients exhibit elevated [[blood pressure]] in a clinical setting but not in other settings.<sup>[[Chronic hypertension differential diagnosis#cite note-pmid24107724-1|[1]]]</sup>   
|Ambulatory blood pressure monitoring and patient self-measurement using a home blood pressure monitoring device are being increasingly used to differentiate patients with white coat hypertension from patients with true hypertension.  
|Ambulatory blood pressure monitoring and patient self-measurement using a home blood pressure monitoring device are being increasingly used to differentiate patients with [[white coat hypertension]] from patients with true hypertension.  
|Normal
|Normal laboratory studies
|-
|-
|Hyperthyroidism
|Hyperthyroidism
|Symptoms of hyperthyroidism include weight loss, heat intolerance, tremor, palpitations, anxiety, increased bowel disturbances, and shortness of breath. <ref name="pmid15963064">{{cite journal| author=Iglesias P, Acosta M, Sánchez R, Fernández-Reyes MJ, Mon C, Díez JJ| title=Ambulatory blood pressure monitoring in patients with hyperthyroidism before and after control of thyroid function. | journal=Clin Endocrinol (Oxf) | year= 2005 | volume= 63 | issue= 1 | pages= 66-72 | pmid=15963064 | doi=10.1111/j.1365-2265.2005.02301.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15963064  }}</ref>
|Symptoms of hyperthyroidism include weight loss, heat intolerance, tremor, palpitations, anxiety, increased bowel disturbances, and shortness of breath. <ref name="pmid15963064">{{cite journal| author=Iglesias P, Acosta M, Sánchez R, Fernández-Reyes MJ, Mon C, Díez JJ| title=Ambulatory blood pressure monitoring in patients with hyperthyroidism before and after control of thyroid function. | journal=Clin Endocrinol (Oxf) | year= 2005 | volume= 63 | issue= 1 | pages= 66-72 | pmid=15963064 | doi=10.1111/j.1365-2265.2005.02301.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15963064  }}</ref>
|Goiter, skin flushing and eye proptosis.
|[[Goiter|Goiter,]] skin flushing and eye proptosis. Increased sensitivity of [[beta receptors]] in the heart to [[catecholamines]]<ref name="pmid20454652">{{cite journal| author=Mintz G, Pizzarello R, Klein I| title=Enhanced left ventricular diastolic function in hyperthyroidism: noninvasive assessment and response to treatment. | journal=J Clin Endocrinol Metab | year= 1991 | volume= 73 | issue= 1 | pages= 146-50 | pmid=2045465 | doi=10.1210/jcem-73-1-146 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2045465  }}</ref> due to an effect of [[Thyroid hormone|thyroid hormones]] increase cardiac work and output and systolic hypertension.<ref name="pmid2045465">{{cite journal| author=Mintz G, Pizzarello R, Klein I| title=Enhanced left ventricular diastolic function in hyperthyroidism: noninvasive assessment and response to treatment. | journal=J Clin Endocrinol Metab | year= 1991 | volume= 73 | issue= 1 | pages= 146-50 | pmid=2045465 | doi=10.1210/jcem-73-1-146 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2045465  }}</ref>
Increased sensitivity of beta receptors in the heart to catecholamines <ref name="pmid20454652">{{cite journal| author=Mintz G, Pizzarello R, Klein I| title=Enhanced left ventricular diastolic function in hyperthyroidism: noninvasive assessment and response to treatment. | journal=J Clin Endocrinol Metab | year= 1991 | volume= 73 | issue= 1 | pages= 146-50 | pmid=2045465 | doi=10.1210/jcem-73-1-146 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2045465  }}</ref> due to an effect of thyroid hormones increase cardiac work and output and systolic hypertension.<ref name="pmid2045465">{{cite journal| author=Mintz G, Pizzarello R, Klein I| title=Enhanced left ventricular diastolic function in hyperthyroidism: noninvasive assessment and response to treatment. | journal=J Clin Endocrinol Metab | year= 1991 | volume= 73 | issue= 1 | pages= 146-50 | pmid=2045465 | doi=10.1210/jcem-73-1-146 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2045465  }}</ref>
|Low [[thyroid-stimulating hormone]] (TSH), high [[Thyroxine|free thyroxine]] (T4), and [[triiodothyronine]] (T3) concentrations
|Low thyroid-stimulating hormone (TSH) high free thyroxine (T4) and triiodothyronine (T3) concentrations.
|-
|-
|Renovascular hypertension
|[[Renovascular hypertension]]
|
|
* Age of [[hypertension]] < 30 years and > 55 years
* Age of [[hypertension]] < 30 years and > 55 years
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* [[Malignant hypertension]]
* [[Malignant hypertension]]
|([[bruit]]) can be heard over the abdomen.
|([[bruit]]) can be heard over the abdomen.
|Diagnosis by Duplex ultrasonography is considered class I recommendation. It may be used as an initial screening tool for diagnosis of atherosclerotic renal artery stenosis. [[Ultrasonography]] might not be very accurate in obese patients or those intestinal gas.<sup>[[Renal artery stenosis ultrasound#cite note-pmid23457117-1|[1]]]</sup>
|Diagnosis by [[Duplex ultrasound|Duplex ultrasonography]] is considered class I recommendation. It may be used as an initial screening tool for diagnosis of atherosclerotic [[renal artery stenosis]]. [[Ultrasonography]] might not be very accurate in obese patients or those intestinal gas.<sup>[[Renal artery stenosis ultrasound#cite note-pmid23457117-1|[1]]]</sup>
|-
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|Stroke and compression of lateral medulla
|[[Stroke]] and [[Lateral medullary syndrome|compression of lateral medulla]] ([[Lateral medullary syndrome]])
|Extensive unilateral infarction of the brain stem in the region of the nucleus tractus solitarius may result in partial baroreflex dysfunction, increased sympathetic activity, and neurogenic paroxysmal hypertension.<ref name="pmid10926969">{{cite journal| author=Phillips AM, Jardine DL, Parkin PJ, Hughes T, Ikram H| title=Brain stem stroke causing baroreflex failure and paroxysmal hypertension. | journal=Stroke | year= 2000 | volume= 31 | issue= 8 | pages= 1997-2001 | pmid=10926969 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10926969  }}</ref>
|Extensive unilateral infarction of the [[brain stem]] in the region of the [[nucleus tractus solitarius]] may result in partial [[Baroreflex|baroreflex dysfunction]], increased sympathetic activity, and neurogenic paroxysmal hypertension.<ref name="pmid10926969">{{cite journal| author=Phillips AM, Jardine DL, Parkin PJ, Hughes T, Ikram H| title=Brain stem stroke causing baroreflex failure and paroxysmal hypertension. | journal=Stroke | year= 2000 | volume= 31 | issue= 8 | pages= 1997-2001 | pmid=10926969 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10926969  }}</ref>
|
|
|CT shows mass compressing lateral medulla or infarction in the same area.
|[[Computed tomography|CT]] shows mass compressing [[Lateral medullary syndrome|lateral medulla]] or infarction in the same area.
|-
|-
|Seizures  
|[[Seizures]]
|According to type:
|According to type; it may be focal or generalized, clinical or subclinical:
* A person having a tonic-clonic seizure may cry out, lose consciousness and fall to the ground, and convulse, often violently.
* A person having a [[tonic-clonic seizure]] may have repetitive twitches of arm and legs. Patients may have tongue pittings and may lose consciousness.
* A person having a [[complex partial seizure]] may appear confused or dazed and will not be able to respond to questions or direction.  
* Symptoms occur suddenly and may persist
* Some people have seizures that are not noticeable to others. Sometimes, the only clue that a person is having an [[absence seizure]] is rapid blinking or a few seconds of staring into space.
* Muscle tension or tightening that causes twisting of the body, head, arms, or legs.
 
* Patients may have [[Amnesia|amnesia.]]
* Change in alertness; the person cannot remember a period of time
* Mood changes for example; fear, panic, or laughter
* Mood changes, such as unexplainable fear, panic, joy, or laughter
* Change in sensation of the skin over the arm, leg, or trunk
* Change in sensation of the skin, usually spreading over the arm, leg, or trunk
* Vision changes and light flashes and may be hallucinations
* Vision changes, including seeing flashing lights
* Rarely, hallucinations (seeing things that aren't there)
* Falling, loss of muscle control occurs very suddenly
* Muscle twitching that may spread up or down an arm or leg
* Muscle tension or tightening that causes twisting of the body, head, arms, or legs
* Shaking of the entire body
* Tasting a bitter or metallic flavor
* Tasting a bitter or metallic flavor
* A person having a [[complex partial seizure]] may appear confused or dazed and will not be able to respond to questions or direction.
* [[absence seizure]] is a rapid blinking or a few seconds of staring into space.
  |
  |
|An isolated abnormal electrical activity recorded by an [[electroencephalography]] examination without a clinical presentation is called sub clinical seizure. They may identify background epileptogenic activity, as well as help identify particular causes of seizures.
|An abnormal [[electroencephalography]]. Positive test without a clinical presentation is called sub clinical seizure. They may identify past or family history of epilepsy.
|-
|-
|[[Carcinoid syndrome]]
|[[Carcinoid syndrome]]
|Hypertensive crisis occurs with malignant carcinoid syndrome <ref name="pmid7969229">{{cite journal| author=Warner RR, Mani S, Profeta J, Grunstein E| title=Octreotide treatment of carcinoid hypertensive crisis. | journal=Mt Sinai J Med | year= 1994 | volume= 61 | issue= 4 | pages= 349-55 | pmid=7969229 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7969229  }}</ref> should be distinguished from pheochromocytoma. Patient 
|Hypertensive crisis occurs with [[malignant carcinoid syndrome]] <ref name="pmid7969229">{{cite journal| author=Warner RR, Mani S, Profeta J, Grunstein E| title=Octreotide treatment of carcinoid hypertensive crisis. | journal=Mt Sinai J Med | year= 1994 | volume= 61 | issue= 4 | pages= 349-55 | pmid=7969229 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7969229  }}</ref> should be distinguished from pheochromocytoma.  
|Cutaneous flushing Venous telangiectasia Diarrhea Bronchospasm Cardiac valvular lesions: tricusped incompeence.
|Cutaneous flushing Venous [[Telangiectasia|telangiectasia,]] diarrhea, [[bronchospasm]] and cardiac valvular lesions ([[Tricuspid regurgitation|tricuspid incompetence]])
|
|
* High urinary excretion of 5-HIAA <ref name="pmid3227292">{{cite journal| author=Sjöblom SM| title=Clinical presentation and prognosis of gastrointestinal carcinoid tumours. | journal=Scand J Gastroenterol | year= 1988 | volume= 23 | issue= 7 | pages= 779-87 | pmid=3227292 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3227292  }}</ref>
* High urinary excretion of [[5-HIAA]] <ref name="pmid3227292">{{cite journal| author=Sjöblom SM| title=Clinical presentation and prognosis of gastrointestinal carcinoid tumours. | journal=Scand J Gastroenterol | year= 1988 | volume= 23 | issue= 7 | pages= 779-87 | pmid=3227292 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3227292  }}</ref>
* High  urinary excretion of serotonin <ref name="pmid2421946">{{cite journal| author=Feldman JM| title=Urinary serotonin in the diagnosis of carcinoid tumors. | journal=Clin Chem | year= 1986 | volume= 32 | issue= 5 | pages= 840-4 | pmid=2421946 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2421946  }}</ref>
* High  urinary excretion of [[serotonin]] <ref name="pmid2421946">{{cite journal| author=Feldman JM| title=Urinary serotonin in the diagnosis of carcinoid tumors. | journal=Clin Chem | year= 1986 | volume= 32 | issue= 5 | pages= 840-4 | pmid=2421946 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2421946  }}</ref>
* High chromogranin concentration Chromogranins (designated as A, B, and C) are proteins that are stored and released with peptides and amines in a variety of neuroendocrine tissues <ref name="pmid2316306">{{cite journal| author=Eriksson B, Arnberg H, Oberg K, Hellman U, Lundqvist G, Wernstedt C et al.| title=A polyclonal antiserum against chromogranin A and B--a new sensitive marker for neuroendocrine tumours. | journal=Acta Endocrinol (Copenh) | year= 1990 | volume= 122 | issue= 2 | pages= 145-55 | pmid=2316306 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2316306  }}</ref>
* High [[chromogranin]] concentration. [[Chromogranin]](designated as A, B, and C) are proteins that are stored and released with [[peptides]] and [[amines]] in a variety of neuroendocrine tissues. <ref name="pmid2316306">{{cite journal| author=Eriksson B, Arnberg H, Oberg K, Hellman U, Lundqvist G, Wernstedt C et al.| title=A polyclonal antiserum against chromogranin A and B--a new sensitive marker for neuroendocrine tumours. | journal=Acta Endocrinol (Copenh) | year= 1990 | volume= 122 | issue= 2 | pages= 145-55 | pmid=2316306 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2316306  }}</ref>
* High  blood serotonin concentration
* High  plasma 5-HIAA concentration


* CT is recommended for evaluation of all patients with carcinoid tumors <ref name="pmid19077417">{{cite journal| author=Sundin A, Vullierme MP, Kaltsas G, Plöckinger U, Mallorca Consensus Conference participants. European Neuroendocrine Tumor Society| title=ENETS Consensus Guidelines for the Standards of Care in Neuroendocrine Tumors: radiological examinations. | journal=Neuroendocrinology | year= 2009 | volume= 90 | issue= 2 | pages= 167-83 | pmid=19077417 | doi=10.1159/000184855 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19077417  }}</ref>
* [[Computed tomography|CT]] is recommended for evaluation of all patients with [[Carcinoid syndrome|carcinoid tumors]].<ref name="pmid19077417">{{cite journal| author=Sundin A, Vullierme MP, Kaltsas G, Plöckinger U, Mallorca Consensus Conference participants. European Neuroendocrine Tumor Society| title=ENETS Consensus Guidelines for the Standards of Care in Neuroendocrine Tumors: radiological examinations. | journal=Neuroendocrinology | year= 2009 | volume= 90 | issue= 2 | pages= 167-83 | pmid=19077417 | doi=10.1159/000184855 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19077417  }}</ref>
|-
|-
|[[Migraine headaches]]   
|[[Migraine headaches]]   
|[[prodrome|(1) '''Prodrome,''']] which occurs hours or days before a headache.
|[[prodrome|(1) '''Prodrome''']] which occurs hours or days before a headache


(2)  '''[[Aura (symptom)|Aura]],''' which immediately precedes the headache.
(2)  '''[[Aura (symptom)|Aura]]''' which immediately precedes the headache  


(3) [[Pain and nociception|'''the pain phase,''']] also known as headache phase and
(3) [[Pain and nociception|The pain phase,]] also known as headache phase  


(4) '''[[Postdrome|The postdrome phase.]]<ref name="pmid15447695">{{cite journal| author=Kelman L| title=The premonitory symptoms (prodrome): a tertiary care study of 893 migraineurs. | journal=Headache | year= 2004 | volume= 44 | issue= 9 | pages= 865-72 | pmid=15447695 | doi=10.1111/j.1526-4610.2004.04168.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15447695  }}</ref>'''
(4) [[Postdrome|The postdrome phase]]'''<ref name="pmid15447695">{{cite journal| author=Kelman L| title=The premonitory symptoms (prodrome): a tertiary care study of 893 migraineurs. | journal=Headache | year= 2004 | volume= 44 | issue= 9 | pages= 865-72 | pmid=15447695 | doi=10.1111/j.1526-4610.2004.04168.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15447695  }}</ref>'''  
|
|
* [[Red eye|Conjunctival injection]] may be present
* [[Red eye|Conjunctival injection]] may be present.


* [[Horner's syndrome]] <sup>[[Migraine physical examination#cite note-1|[1]]]</sup> may be present
* [[Horner's syndrome]] <sup>[[Migraine physical examination#cite note-1|[1]]]</sup> may be present.
* [[Adie syndrome|Adie type pupil]] <sup>[[Migraine physical examination#cite note-2|[2]]]</sup> may be present
* [[Adie syndrome|Adie type pupil]] <sup>[[Migraine physical examination#cite note-2|[2]]]</sup> may be present.


* Cranial/ cervical muscle [[tenderness]] may be present
* Cranial/ cervical muscle [[tenderness]] may be present.
* Listen for bruit at neck and head for clinical sights of [[Cerebral arteriovenous malformation|arteriovenous malformation]].
* Listen for bruit at neck and head for clinical sights of [[Cerebral arteriovenous malformation|an arteriovenous malformation]].
|'''[[CT]] is indicated in patients with: <sup>[[Migraine CT#cite note-1|[1]]]</sup> <sup>[[Migraine CT#cite note-pmid24400971-2|[2]]]</sup>'''
|'''[[CT]] is indicated in patients with<sup>[[Migraine CT#cite note-1|[1]]]</sup> <sup>[[Migraine CT#cite note-pmid24400971-2|[2]]]</sup>'''
* Abnormal [[physical examination]]
* Abnormal [[physical examination]]
** Increase of [[headache]]'s frequency
** Increase of [[headache]]'s frequency
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** [[Headache]]'s awakening the patient at night<sup>[[Migraine CT#cite note-3|[3]]][[Migraine CT#cite note-4|[4]]]</sup>
** [[Headache]]'s awakening the patient at night<sup>[[Migraine CT#cite note-3|[3]]][[Migraine CT#cite note-4|[4]]]</sup>
* Atypical [[aura]]: sudden onset, lasting more than 1 hour, always at the same side and/or without [[visual]] symptoms
* Atypical [[aura]]: sudden onset, lasting more than 1 hour, always at the same side and/or without [[visual]] symptoms
* Migraine attacks that begin after 50 years of age
* [[Migraine]] attacks that begin after 50 years of age
'''[[CT]] is not indicated in:'''
'''[[CT]] is not indicated in:'''
* Patients with a diagnosis of a migraine in accordance with the [[Migraine classification|criteria for migraine]].
* Patients with a diagnosis of a migraine in accordance with the [[Migraine classification|criteria for migraine]]
* Differentiating a migraine from other primary [[headaches]]
* Differentiating a migraine from other primary [[headaches]]
|-
|-
|Drugs
|Drugs
|Sympathomimetic drugs that can induce symptoms simulating pheochromocytoma include high-dose phenylpropanolamin, cocaine, amphetamine, LSD, PCP. <ref name="pmid11358774">{{cite journal| author=Krentz AJ, Mikhail S, Cantrell P, Hill GM| title=Drug Points: Pseudophaeochromocytoma syndrome associated with clozapine. | journal=BMJ | year= 2001 | volume= 322 | issue= 7296 | pages= 1213 | pmid=11358774 | doi= | pmc=31620 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11358774  }}</ref> Combination of a monoamine oxidase (MAO) inhibitor and ingestion of tyramine-containing foods.<ref name="pmid3980057">{{cite journal| author=Kuchel O| title=Pseudopheochromocytoma. | journal=Hypertension | year= 1985 | volume= 7 | issue= 1 | pages= 151-8 | pmid=3980057 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3980057  }}</ref>
|[[Sympathomimetic drug|Sympathomimetic drugs]] that can induce symptoms simulating pheochromocytoma include high-dose [[phenylpropanolamine]], [[cocaine]], [[amphetamine]], [[LSD]], [[PCP|PCP.]] <ref name="pmid11358774">{{cite journal| author=Krentz AJ, Mikhail S, Cantrell P, Hill GM| title=Drug Points: Pseudophaeochromocytoma syndrome associated with clozapine. | journal=BMJ | year= 2001 | volume= 322 | issue= 7296 | pages= 1213 | pmid=11358774 | doi= | pmc=31620 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11358774  }}</ref> Combination of a [[Monoamine oxidase inhibitor|monoamine oxidase (MAO) inhibitor]] and ingestion of [[Tyramine|tyramine-containing]] foods.<ref name="pmid3980057">{{cite journal| author=Kuchel O| title=Pseudopheochromocytoma. | journal=Hypertension | year= 1985 | volume= 7 | issue= 1 | pages= 151-8 | pmid=3980057 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3980057  }}</ref>
|
|
* Disturbed consciousness.
* Disturbed consciousness
* nasal septum perforation in cocaine addiction.
* Nasal septum perforation in [[cocaine addiction]]
* Needle marks in the skin.
* Needle marks on the skin
* History of antidepressants intake.
* History of [[antidepressants]] intake
|Toxicology screening
|[[Toxicology screen|Toxicology screening]]
|-
|-
|Baroreflex failure
|[[Baroreflex|Baroreflex failure]]
|
|
* Marked and frequent fluctuations in blood pressure, <ref name="pmid183225442">{{cite journal| author=Zar T, Peixoto AJ| title=Paroxysmal hypertension due to baroreflex failure. | journal=Kidney Int | year= 2008 | volume= 74 | issue= 1 | pages= 126-31 | pmid=18322544 | doi=10.1038/ki.2008.30 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18322544  }}</ref>with both high and low readings .
* Marked and frequent fluctuations in [[blood pressure]], <ref name="pmid183225442">{{cite journal| author=Zar T, Peixoto AJ| title=Paroxysmal hypertension due to baroreflex failure. | journal=Kidney Int | year= 2008 | volume= 74 | issue= 1 | pages= 126-31 | pmid=18322544 | doi=10.1038/ki.2008.30 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18322544  }}</ref>with both high and low readings  
* It is caused by hypofunctioning of the baroreflexes that normally buffer blood pressure fluctuations.  
* It is caused by hypofunctioning of [[Baroreflex|t-he baroreflexes]] that normally buffer blood pressure fluctuations.  
* The disorder is usually a result of injury to carotid baroreceptors, with most patients reporting a history of neck irradiation or surgery. <ref name="pmid18322544">{{cite journal| author=Zar T, Peixoto AJ| title=Paroxysmal hypertension due to baroreflex failure. | journal=Kidney Int | year= 2008 | volume= 74 | issue= 1 | pages= 126-31 | pmid=18322544 | doi=10.1038/ki.2008.30 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18322544  }}</ref>
* The disorder is usually a result of injury to [[Baroreceptors|carotid baroreceptors]], with most patients reporting a history of neck irradiation or surgery. <ref name="pmid18322544">{{cite journal| author=Zar T, Peixoto AJ| title=Paroxysmal hypertension due to baroreflex failure. | journal=Kidney Int | year= 2008 | volume= 74 | issue= 1 | pages= 126-31 | pmid=18322544 | doi=10.1038/ki.2008.30 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18322544  }}</ref>
|
|
|Neck CT
|Neck [[Computed tomography|CT]]
|}   
|}   


Revision as of 16:46, 31 July 2017

Pheochromocytoma Microchapters

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ahmad Al Maradni, M.D. [2] Mohammed Abdelwahed M.D[3]

Overview

Pheochromocytoma must be differentiated from other causes of paroxysmal hypertension including severe paroxysmal hypertension (Pseudopheochromocytoma)

, panic disorder, Factitious hypertension, carcinoid syndrome, Migraine headache, Hyperthyroidism, Renovascular hypertension, Hypoglycemia, Labile hypertension (White coat hypertension), Stroke and compression of the lateral medulla, Seizures, Baroreflex failure and drugs.

Differentiating pheochromocytoma from other diseases

Pheochromocytoma must be differentiated from other causes of paroxysmal hypertension including severe paroxysmal hypertension (Pseudopheochromocytoma), panic disorder, Factitious hypertension, carcinoid syndrome, Migraine headache, Hyperthyroidism, Renovascular hypertension, Hypoglycemia and drugs.

Disease Symptoms Signs Investigations
Pheochromocytoma The hallmark symptoms of a pheochromocytoma are those of sympathetic nervous system hyperactivity, symptoms usually subside in less than one hour and they may include:

Please note that not all patients with pheochromocytoma experience all classical symptoms.[4]

Common physical exam findings include:

High-risk patients: plasma fractionated metanephrines are the first test if elevated; 24-hour urinary fractionated metanephrines, catecholamines, and imaging should be the second test for diagnosis. [2]

Low-risk patients: 24-hour urinary fractionated catecholamines and metanephrines.[4]

Pseudopheochromocytoma Paroxysmal activation of the sympathetic system causing hypertension and tachycardia [1]that responds to alpha/beta blockade.[2]Disorder is usually related to emotional distress.

Acute onset of high blood pressure, headache, chest pain, nausea, palpitations, flushing. Duration of attacks ranges from minutes to hours.

Increase in plasma catecholamines between and during attacks.[3][4]
Panic attacks
  • Paroxysms of increased sympathetic activity.
  • It is characterized by episodes of fear or panic
  • Such as pseudo-pheochromocytoma, panic disorders are usually associated with physical symptoms such as chest pain, headache, palpitations, flushing and both respond to antidepressants.
 In panic attacks, fear precedes physical symptoms. By contrast, in pseudo pheochromocytoma physical symptoms occur before feeling fear.

Laboratory studies that can exclude medical disorders other than panic disorder include:

Labile hypertension (White coat hypertension) Patients exhibit elevated blood pressure in a clinical setting but not in other settings.[1]  Ambulatory blood pressure monitoring and patient self-measurement using a home blood pressure monitoring device are being increasingly used to differentiate patients with white coat hypertension from patients with true hypertension. Normal laboratory studies
Hyperthyroidism Symptoms of hyperthyroidism include weight loss, heat intolerance, tremor, palpitations, anxiety, increased bowel disturbances, and shortness of breath. [5] Goiter, skin flushing and eye proptosis. Increased sensitivity of beta receptors in the heart to catecholamines[6] due to an effect of thyroid hormones increase cardiac work and output and systolic hypertension.[7] Low thyroid-stimulating hormone (TSH), high free thyroxine (T4), and triiodothyronine (T3) concentrations
Renovascular hypertension (bruit) can be heard over the abdomen. Diagnosis by Duplex ultrasonography is considered class I recommendation. It may be used as an initial screening tool for diagnosis of atherosclerotic renal artery stenosis. Ultrasonography might not be very accurate in obese patients or those intestinal gas.[1]
Stroke and compression of lateral medulla (Lateral medullary syndrome) Extensive unilateral infarction of the brain stem in the region of the nucleus tractus solitarius may result in partial baroreflex dysfunction, increased sympathetic activity, and neurogenic paroxysmal hypertension.[8] CT shows mass compressing lateral medulla or infarction in the same area.
Seizures According to type; it may be focal or generalized, clinical or subclinical:
  • A person having a tonic-clonic seizure may have repetitive twitches of arm and legs. Patients may have tongue pittings and may lose consciousness.
  • Symptoms occur suddenly and may persist
  • Muscle tension or tightening that causes twisting of the body, head, arms, or legs.
  • Patients may have amnesia.
  • Mood changes for example; fear, panic, or laughter
  • Change in sensation of the skin over the arm, leg, or trunk
  • Vision changes and light flashes and may be hallucinations
  • Tasting a bitter or metallic flavor
  • A person having a complex partial seizure may appear confused or dazed and will not be able to respond to questions or direction.
  • absence seizure is a rapid blinking or a few seconds of staring into space.
 An abnormal electroencephalography. Positive test without a clinical presentation is called sub clinical seizure. They may identify past or family history of epilepsy.
Carcinoid syndrome Hypertensive crisis occurs with malignant carcinoid syndrome [9] should be distinguished from pheochromocytoma. Cutaneous flushing Venous telangiectasia, diarrhea, bronchospasm and cardiac valvular lesions (tricuspid incompetence)
Migraine headaches (1) Prodrome which occurs hours or days before a headache

(2)  Aura which immediately precedes the headache

(3) The pain phase, also known as headache phase

(4) The postdrome phase[14]

CT is indicated in patients with[1] [2]

CT is not indicated in:

Drugs Sympathomimetic drugs that can induce symptoms simulating pheochromocytoma include high-dose phenylpropanolamine, cocaine, amphetamine, LSD, PCP. [15] Combination of a monoamine oxidase (MAO) inhibitor and ingestion of tyramine-containing foods.[16] Toxicology screening
Baroreflex failure
  • Marked and frequent fluctuations in blood pressure, [17]with both high and low readings
  • It is caused by hypofunctioning of t-he baroreflexes that normally buffer blood pressure fluctuations.
  • The disorder is usually a result of injury to carotid baroreceptors, with most patients reporting a history of neck irradiation or surgery. [18]
 Neck CT
  1. ↑ Mann SJ (1999). "Severe paroxysmal hypertension (pseudopheochromocytoma): understanding the cause and treatment". Arch Intern Med. 159 (7): 670–4. PMID 10218745.
  2. ↑ Mann SJ (1999). "Severe paroxysmal hypertension (pseudopheochromocytoma): understanding the cause and treatment". Arch Intern Med. 159 (7): 670–4. PMID 10218745.
  3. ↑ Mann SJ (1996). "Severe paroxysmal hypertension. An automatic syndrome and its relationship to repressed emotions". Psychosomatics. 37 (5): 444–50. doi:10.1016/S0033-3182(96)71532-3. PMID 8824124.
  4. ↑ Sharabi Y, Goldstein DS, Bentho O, Saleem A, Pechnik S, Geraci MF; et al. (2007). "Sympathoadrenal function in patients with paroxysmal hypertension: pseudopheochromocytoma". J Hypertens. 25 (11): 2286–95. doi:10.1097/HJH.0b013e3282ef5fac. PMID 17921824.
  5. ↑ Iglesias P, Acosta M, Sánchez R, Fernández-Reyes MJ, Mon C, Díez JJ (2005). "Ambulatory blood pressure monitoring in patients with hyperthyroidism before and after control of thyroid function". Clin Endocrinol (Oxf). 63 (1): 66–72. doi:10.1111/j.1365-2265.2005.02301.x. PMID 15963064.
  6. ↑ Mintz G, Pizzarello R, Klein I (1991). "Enhanced left ventricular diastolic function in hyperthyroidism: noninvasive assessment and response to treatment". J Clin Endocrinol Metab. 73 (1): 146–50. doi:10.1210/jcem-73-1-146. PMID 2045465.
  7. ↑ Mintz G, Pizzarello R, Klein I (1991). "Enhanced left ventricular diastolic function in hyperthyroidism: noninvasive assessment and response to treatment". J Clin Endocrinol Metab. 73 (1): 146–50. doi:10.1210/jcem-73-1-146. PMID 2045465.
  8. ↑ Phillips AM, Jardine DL, Parkin PJ, Hughes T, Ikram H (2000). "Brain stem stroke causing baroreflex failure and paroxysmal hypertension". Stroke. 31 (8): 1997–2001. PMID 10926969.
  9. ↑ Warner RR, Mani S, Profeta J, Grunstein E (1994). "Octreotide treatment of carcinoid hypertensive crisis". Mt Sinai J Med. 61 (4): 349–55. PMID 7969229.
  10. ↑ Sjöblom SM (1988). "Clinical presentation and prognosis of gastrointestinal carcinoid tumours". Scand J Gastroenterol. 23 (7): 779–87. PMID 3227292.
  11. ↑ Feldman JM (1986). "Urinary serotonin in the diagnosis of carcinoid tumors". Clin Chem. 32 (5): 840–4. PMID 2421946.
  12. ↑ Eriksson B, Arnberg H, Oberg K, Hellman U, Lundqvist G, Wernstedt C; et al. (1990). "A polyclonal antiserum against chromogranin A and B--a new sensitive marker for neuroendocrine tumours". Acta Endocrinol (Copenh). 122 (2): 145–55. PMID 2316306.
  13. ↑ Sundin A, Vullierme MP, Kaltsas G, Plöckinger U, Mallorca Consensus Conference participants. European Neuroendocrine Tumor Society (2009). "ENETS Consensus Guidelines for the Standards of Care in Neuroendocrine Tumors: radiological examinations". Neuroendocrinology. 90 (2): 167–83. doi:10.1159/000184855. PMID 19077417.
  14. ↑ Kelman L (2004). "The premonitory symptoms (prodrome): a tertiary care study of 893 migraineurs". Headache. 44 (9): 865–72. doi:10.1111/j.1526-4610.2004.04168.x. PMID 15447695.
  15. ↑ Krentz AJ, Mikhail S, Cantrell P, Hill GM (2001). "Drug Points: Pseudophaeochromocytoma syndrome associated with clozapine". BMJ. 322 (7296): 1213. PMC 31620. PMID 11358774.
  16. ↑ Kuchel O (1985). "Pseudopheochromocytoma". Hypertension. 7 (1): 151–8. PMID 3980057.
  17. ↑ Zar T, Peixoto AJ (2008). "Paroxysmal hypertension due to baroreflex failure". Kidney Int. 74 (1): 126–31. doi:10.1038/ki.2008.30. PMID 18322544.
  18. ↑ Zar T, Peixoto AJ (2008). "Paroxysmal hypertension due to baroreflex failure". Kidney Int. 74 (1): 126–31. doi:10.1038/ki.2008.30. PMID 18322544.
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