Pheochromocytoma laboratory findings: Difference between revisions

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==Laboratory Findings==
==Laboratory Findings==
Diagnostic lab findings associated with pheochromocytoma include:
An elevated concentration of the following is diagnostic of [[pheochromocytoma]].
* Elevated [[plasma]] and [[urinary]] [[catecholamine]]s and [[metanephrine]]s
* Elevated [[plasma]] and [[urinary]] [[catecholamine]]s and [[Plasma]] fractionated [[Metanephrine|metanephrines]] levels  
* Elevated urinary [[vanillyl mandelic acid]]
* Elevated urinary [[vanillyl mandelic acid] <ref name="pmid11903030">{{cite journal| author=Lenders JW, Pacak K, Walther MM, Linehan WM, Mannelli M, Friberg P et al.| title=Biochemical diagnosis of pheochromocytoma: which test is best? | journal=JAMA | year= 2002 | volume= 287 | issue= 11 | pages= 1427-34 | pmid=11903030 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11903030  }}</ref>
'''Indications of pheochromocytoma testing''':<ref name="pmid17121518">{{cite journal| author=Gimenez-Roqueplo AP, Lehnert H, Mannelli M, Neumann H, Opocher G, Maher ER et al.| title=Phaeochromocytoma, new genes and screening strategies. | journal=Clin Endocrinol (Oxf) | year= 2006 | volume= 65 | issue= 6 | pages= 699-705 | pmid=17121518 | doi=10.1111/j.1365-2265.2006.02714.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17121518  }}</ref>
* Triad of [[tachycardia]], [[headache]], and [[sweating]].
* Episodes of [[palpitation]], [[headache]] and [[tremors]] for unknown reasons.
* [[Hypertension]] at age <20 years, resistant [[hypertension]].
* A [[family history]] of pheochromocytoma, [[multiple endocrine neoplasia]] [[Multiple endocrine neoplasia type 1|types 1]] and [[MEN2b|2B]], [[neurofibromatosis type 1]], or [[Von Hippel-Lindau disease]].
* The presence of [[bilateral]], extra-[[Adrenal gland|adrenal]] or multiple [[Tumor|tumors]] or a [[Malignant tumors|malignant tumor]], should be seen as indications for [[genetic testing]].
* An [[Incidentaloma|incidentally]] discovered [[Adrenal mass causes|adrenal mass]] that does not have imaging characteristics consistent with pheochromocytoma.
'''High-risk patients'''
* [[Plasma]] fractionated [[Metanephrine|metanephrines]] levels is the first test and if elevated, 24-hour [[urinary]] fractionated [[Metanephrine|metanephrines]], [[catecholamines]], and imaging should be the second test for the purpose of diagnosis.<ref name="pmid11903030">{{cite journal| author=Lenders JW, Pacak K, Walther MM, Linehan WM, Mannelli M, Friberg P et al.| title=Biochemical diagnosis of pheochromocytoma: which test is best? | journal=JAMA | year= 2002 | volume= 287 | issue= 11 | pages= 1427-34 | pmid=11903030 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11903030  }}</ref>
* High-risk patients include a [[family history]] of  [[MEN2]] and [[Von Hippel-Lindau disease|VHL]] syndrome or past history of pheochromocytoma.
* Diagnostic cutoffs to exclude pheochromocytoma are [[metanephrine]] <0.3 nmol/L and [[normetanephrine]] <0.66 nmol/L.<ref name="pmid7778821">{{cite journal| author=Lenders JW, Keiser HR, Goldstein DS, Willemsen JJ, Friberg P, Jacobs MC et al.| title=Plasma metanephrines in the diagnosis of pheochromocytoma. | journal=Ann Intern Med | year= 1995 | volume= 123 | issue= 2 | pages= 101-9 | pmid=7778821 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7778821  }}</ref>
'''Low-risk patients'''


Twenty-four hour [[urinary]] fractionated [[catecholamines]] and [[Metanephrine|metanephrines]] are the tests of choice. The cut-off values are as follows:<ref name="pmid12574179">{{cite journal| author=Sawka AM, Jaeschke R, Singh RJ, Young WF| title=A comparison of biochemical tests for pheochromocytoma: measurement of fractionated plasma metanephrines compared with the combination of 24-hour urinary metanephrines and catecholamines. | journal=J Clin Endocrinol Metab | year= 2003 | volume= 88 | issue= 2 | pages= 553-8 | pmid=12574179 | doi=10.1210/jc.2002-021251 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12574179  }}</ref>
* Diagnostic cutoffs to exclude pheochromocytoma are:
**[[Metanephrine]] <0.3 nmol/L 
** [[Normetanephrine]] <0.66 nmol/L. <ref name="pmid7778821">{{cite journal| author=Lenders JW, Keiser HR, Goldstein DS, Willemsen JJ, Friberg P, Jacobs MC et al.| title=Plasma metanephrines in the diagnosis of pheochromocytoma. | journal=Ann Intern Med | year= 1995 | volume= 123 | issue= 2 | pages= 101-9 | pmid=7778821 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7778821  }}</ref>
 
The cut-off values are as follows:<ref name="pmid12574179">{{cite journal| author=Sawka AM, Jaeschke R, Singh RJ, Young WF| title=A comparison of biochemical tests for pheochromocytoma: measurement of fractionated plasma metanephrines compared with the combination of 24-hour urinary metanephrines and catecholamines. | journal=J Clin Endocrinol Metab | year= 2003 | volume= 88 | issue= 2 | pages= 553-8 | pmid=12574179 | doi=10.1210/jc.2002-021251 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12574179  }}</ref>
* [[Normetanephrine]] >900 mcg/24 hours
* [[Normetanephrine]] >900 mcg/24 hours
* [[Metanephrine]] >400 mcg/24 hours
* [[Metanephrine]] >400 mcg/24 hours
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* [[Dopamine]] >700 mcg/24 hours
* [[Dopamine]] >700 mcg/24 hours


* No further evaluation is necessary if above mentioned results are negative.
Discontinue [[Tricyclic antidepressant|TCAs]] two weeks before any [[hormonal]] assessments because they may affect [[urinary]] [[catecholamines]] metabolism.<ref name="pmid171215182">{{cite journal| author=Gimenez-Roqueplo AP, Lehnert H, Mannelli M, Neumann H, Opocher G, Maher ER et al.| title=Phaeochromocytoma, new genes and screening strategies. | journal=Clin Endocrinol (Oxf) | year= 2006 | volume= 65 | issue= 6 | pages= 699-705 | pmid=17121518 | doi=10.1111/j.1365-2265.2006.02714.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17121518  }}</ref>
'''NB''': Discontinue [[Tricyclic antidepressant|TCAs]] two weeks before any [[hormonal]] assessments because they may affect [[urinary]] [[catecholamines]] metabolism.<ref name="pmid171215182">{{cite journal| author=Gimenez-Roqueplo AP, Lehnert H, Mannelli M, Neumann H, Opocher G, Maher ER et al.| title=Phaeochromocytoma, new genes and screening strategies. | journal=Clin Endocrinol (Oxf) | year= 2006 | volume= 65 | issue= 6 | pages= 699-705 | pmid=17121518 | doi=10.1111/j.1365-2265.2006.02714.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17121518  }}</ref>


Patients with spells of elevated [[blood pressure]] (sudden onset of a [[symptom]] or [[symptoms]]) can be negative in-between spells and should be tested directly after the attacks.<ref name="pmid7630214">{{cite journal| author=Young WF, Maddox DE| title=Spells: in search of a cause. | journal=Mayo Clin Proc | year= 1995 | volume= 70 | issue= 8 | pages= 757-65 | pmid=7630214 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7630214  }}</ref>
Patients with spells of elevated [[blood pressure]] (sudden onset of a [[symptom]] or [[symptoms]]) can be negative in-between spells and should be tested directly after the attacks.<ref name="pmid7630214">{{cite journal| author=Young WF, Maddox DE| title=Spells: in search of a cause. | journal=Mayo Clin Proc | year= 1995 | volume= 70 | issue= 8 | pages= 757-65 | pmid=7630214 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7630214  }}</ref>


'''Genetic testing:'''
It is suggested for:
*[[Bilateral]] adrenal pheochromocytoma
* A [[family history]] of  [[Von Hippel-Lindau disease|Von Hippel-Lindau syndrome]], [[Multiple endocrine neoplasia type 2|MEN2]] and [[Neurofibromatosis type I|neurofibromatosis type 1]]
* [[Paraganglioma]]
* Unilateral pheochromocytoma at a young age
==References==
==References==
{{Reflist|2}}
{{Reflist|2}}


[[Category:Endocrinology]]
[[Category:Endocrinology]]

Revision as of 21:21, 28 July 2020

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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

Laboratory findings of pheochromocytoma include elevated 24-hour urinary fractionated catecholamines and metanephrines for low-risk patients and plasma fractionated metanephrines for high-risk ones.

Laboratory Findings

An elevated concentration of the following is diagnostic of pheochromocytoma.

The cut-off values are as follows:[3]

Discontinue TCAs two weeks before any hormonal assessments because they may affect urinary catecholamines metabolism.[4]

Patients with spells of elevated blood pressure (sudden onset of a symptom or symptoms) can be negative in-between spells and should be tested directly after the attacks.[5]

References

  1. ↑ Lenders JW, Pacak K, Walther MM, Linehan WM, Mannelli M, Friberg P; et al. (2002). "Biochemical diagnosis of pheochromocytoma: which test is best?". JAMA. 287 (11): 1427–34. PMID 11903030.
  2. ↑ Lenders JW, Keiser HR, Goldstein DS, Willemsen JJ, Friberg P, Jacobs MC; et al. (1995). "Plasma metanephrines in the diagnosis of pheochromocytoma". Ann Intern Med. 123 (2): 101–9. PMID 7778821.
  3. ↑ Sawka AM, Jaeschke R, Singh RJ, Young WF (2003). "A comparison of biochemical tests for pheochromocytoma: measurement of fractionated plasma metanephrines compared with the combination of 24-hour urinary metanephrines and catecholamines". J Clin Endocrinol Metab. 88 (2): 553–8. doi:10.1210/jc.2002-021251. PMID 12574179.
  4. ↑ Gimenez-Roqueplo AP, Lehnert H, Mannelli M, Neumann H, Opocher G, Maher ER; et al. (2006). "Phaeochromocytoma, new genes and screening strategies". Clin Endocrinol (Oxf). 65 (6): 699–705. doi:10.1111/j.1365-2265.2006.02714.x. PMID 17121518.
  5. ↑ Young WF, Maddox DE (1995). "Spells: in search of a cause". Mayo Clin Proc. 70 (8): 757–65. PMID 7630214.
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