Pheochromocytoma differential diagnosis: Difference between revisions

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|'''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:
* [[Palpitations]] especially in epinephrine producing tumors.<sup>[[Pheochromocytoma history and symptoms#cite note-pmid8325290-3|[3]]]</sup>
* [[Anxiety]] often resembling that of a [[panic attack]]
* [[Sweating]]
* [[Headaches]]: occurs in 90 % of patients.
* paroxysmal attacks of [[hypertension]] but some patients have normal blood pressure.
* May be asymptomatic and discovered by incidence screening especially [[MEN, type 2|MEN]] patients.
''Please note that not all patients with pheochromocytoma experience all classical symptoms''.<sup>[[Pheochromocytoma history and symptoms#cite note-pmid151327242-4|[4]]]</sup>
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Revision as of 19:41, 6 July 2017

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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]

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, Insulinoma, 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]

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
  • Paroxysm of increased sympathetic activity.
  • Characterized by episodes of fear or panic
  • Such as psudopheochromocytoma, 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 pseudopheochromocytoma physical symptoms occur before feeling fear.

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
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 heart to catecholamines [6] due to 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 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 Symptoms experienced by a person during a seizure depend on where in the brain the disturbance in electrical activity occurs. Recent studies show that seizures happen in sleep more often than was thought. 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 complex partial seizure may appear confused or dazed and will not be able to respond to questions or direction. 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.
  • Change in alertness; the person cannot remember a period of time
  • Mood changes, such as unexplainable fear, panic, joy, or laughter
  • Change in sensation of the skin, usually spreading over the arm, leg, or trunk
  • 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

Many seizures, especially in children, are preceded by tachycardia that frequently persists throughout the seizure. This early increase in heart rate may supplement an aura as a physiological warning sign of an imminent seizure.

An isolated abnormal electrical activity recorded by an electroencephalography examination without a clinical presentation is called subclinical seizure. They may identify background epileptogenic activity, as well as help identify particular causes of seizures.
Carcinoid syndrome Hypertensive crisis occurs with malignant carcinoid syndrome [9] should be distinguished from pheochromocytoma. Patient Cutaneous flushing Venous telangiectasia Diarrhea Bronchospasm Cardiac valvular lesions : tricusped incompitence.
  • High urinary excretion of 5-HIAA [10]
  • High urinary excretion of serotonin [11]
  • 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 [12]
  • High blood serotonin concentration
  • High plasma 5-HIAA concentration
  • CT is recommended for evaluation of all patients with carcinoid tumors [13]
Migraine headaches (1) Prodrome, which occurs hours or days before the headache,

(2)  Aura, which immediately precedes the headache,

(3) the pain phase, also known as headache phase and

(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 phenylpropanolamin, cocaine, amphetamine, LSD, PCP. [15] Combination of a monoamine oxidase (MAO) inhibitor and ingestion of tyramine-containing foods.[16]
  • Disturbed consciousness.
  • nasal septum perforation in cocaine addiction.
  • Needle marks in skin.
  • History of antidepressants intake.
Toxicology screening
Baroreflex failure
  • Marked and frequent fluctuations in blood pressure, [17]with both high and low readings .
  • It is caused by hypofunctioning of the 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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