|
|
Line 4: |
Line 4: |
|
| |
|
| ==Overview== | | ==Overview== |
| Effective measures for the primary prevention of [[pheochromocytoma]] include [[biochemical]] [[Screening (medicine)|screening]] for family members of [[MEN2]] patients is mandatory and [[Genetic]] testing in first-degree relatives of a patient with proven [[germline]] ''[[RET proto-oncogene|RET]]'' [[mutation]].
| | There are no established measures for the primary prevention of [[pheochromocytoma]]. |
|
| |
|
| ==Primary Prevention== | | ==Primary Prevention== |
| ===Biochemical screening===
| | There are no established measures for the primary prevention of [[pheochromocytoma]]. |
| * According to the Endocrine Society, [[biochemical]] [[Screening (medicine)|screening]] for pheochromocytoma in recommended among patients with:
| |
| ** [[Von Hippel-Lindau tumor suppressor|VHL syndrome]]- started at 5 years of age with [[biochemical]] surveillance every year for the rest of life.
| |
| ** Signs or symptoms suggesting catecholamine excess, especially if the symptoms are paroxysmal.
| |
| ** Unexpected blood pressure changes to drugs, surgery, or anesthesia
| |
| ** Unexplained blood pressure variability
| |
| ** Incidentaloma, even if the patient is normotensive
| |
| ** Blood pressure that is difficult to control
| |
| ** History of previous treatment for pheochromocytoma or paraganglioma
| |
| ** Hereditary risk of pheochromocytoma or paraganglioma in family members
| |
| ** Syndromic features relating to a pheochromocytoma-related hereditary syndromes <ref name="pmid24893135">{{cite journal| author=Lenders JW, Duh QY, Eisenhofer G, Gimenez-Roqueplo AP, Grebe SK, Murad MH | display-authors=etal| title=Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline. | journal=J Clin Endocrinol Metab | year= 2014 | volume= 99 | issue= 6 | pages= 1915-42 | pmid=24893135 | doi=10.1210/jc.2014-1498 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24893135 }} </ref>
| |
| *[[Plasma]] fractionated [[metanephrine]] level is the best test. If elevated, 24-hour [[urinary]] fractionated [[Metanephrine|metanephrines]] should be done.
| |
| | |
| ===Imaging screening===
| |
| [[Anatomic]] imaging should be used when [[norepinephrine]] levels are elevated more than two times upper normal limits.<ref name="pmid26451910">{{cite journal| author=Aufforth RD, Ramakant P, Sadowski SM, Mehta A, Trebska-McGowan K, Nilubol N et al.| title=Pheochromocytoma Screening Initiation and Frequency in von Hippel-Lindau Syndrome. | journal=J Clin Endocrinol Metab | year= 2015 | volume= 100 | issue= 12 | pages= 4498-504 | pmid=26451910 | doi=10.1210/jc.2015-3045 | pmc=4667160 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26451910 }}</ref>
| |
| * For high-risk children, [[Screening (medicine)|screening]] for pheochromocytoma should begin by 11 years of age.
| |
| * For moderate risk patients, [[Screening (medicine)|screening]] should be started by 16 years of age.
| |
| * If positive, [[Adrenal gland|adrenal]] imaging ([[Computed tomography|CT]]) or ([[Magnetic resonance imaging|MRI]]) should be performed.
| |
| | |
| === Genetic screening ===
| |
| * Genetic testing should be performed in:<ref name="pmid24893135">{{cite journal| author=Lenders JW, Duh QY, Eisenhofer G, Gimenez-Roqueplo AP, Grebe SK, Murad MH et al.| title=Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline. | journal=J Clin Endocrinol Metab | year= 2014 | volume= 99 | issue= 6 | pages= 1915-42 | pmid=24893135 | doi=10.1210/jc.2014-1498 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24893135 }}</ref>
| |
| ** Patients with a [[family history]] of pheochromocytoma
| |
| **[[Tumors]] or [[malignant]] or extra-[[Adrenal gland|adrenal]] pheochromocytoma
| |
| ** Families whose infants or young children have [[Hirschsprung's disease|Hirschsprung disease]]
| |
| **[[First degree relative|First-degree relatives]] of a patient with proven [[Germline mutation|germline]] ''[[RET proto-oncogene|RET]]'' [[mutation]]
| |
| ** Patients with [[cutaneous]] lichen [[amyloidosis]]
| |
| ** Patients with known ''[[RET proto-oncogene|RET]]'' mutations.
| |
| ** Parents whose young children have [[Multiple endocrine neoplasia type 2|MEN 2A/2B]]
| |
| | |
| == References ==
| |
| {{reflist|2}}
| |