Pheochromocytoma screening: Difference between revisions
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Biochemical screening for pheochromocytoma in pediatric patients with VHL starting at 5 years of age with lifelong biochemical surveillance every year and the use of anatomic imaging when normetanephrine levels are elevated more than two times the ULN.<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> | Biochemical screening for pheochromocytoma in pediatric patients with VHL starting at 5 years of age with lifelong biochemical surveillance every year and the use of anatomic imaging when normetanephrine levels are elevated more than two times the ULN.<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> | ||
Genetic testing should be performed in: | |||
* Patients with a family history of pheochromocytoma, | |||
* Bilateral or multifocal lesions. | |||
* tumors or malignant or extra-adrenal pheochromocytoma, | |||
* Young patients who are aged 50 years or under. | |||
==References== | ==References== | ||
Revision as of 14:55, 7 July 2017
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Pheochromocytoma Microchapters |
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Overview
Screening
Familial pheochromocytoma is associated with many syndromes. Multiple endocrien neoplasia (MEN2) is one of them. Pheochromocytoma occurs in 50% of patients with MEN2. which is autosomal dominant syndromes controlled by RET gene. Biochemical screening for family members of MEN2 patients is mandatory.
Biochemical screening for pheochromocytoma in pediatric patients with VHL starting at 5 years of age with lifelong biochemical surveillance every year and the use of anatomic imaging when normetanephrine levels are elevated more than two times the ULN.[1]
Genetic testing should be performed in:
- Patients with a family history of pheochromocytoma,
- Bilateral or multifocal lesions.
- tumors or malignant or extra-adrenal pheochromocytoma,
- Young patients who are aged 50 years or under.
References
- ↑ Aufforth RD, Ramakant P, Sadowski SM, Mehta A, Trebska-McGowan K, Nilubol N; et al. (2015). "Pheochromocytoma Screening Initiation and Frequency in von Hippel-Lindau Syndrome". J Clin Endocrinol Metab. 100 (12): 4498–504. doi:10.1210/jc.2015-3045. PMC 4667160. PMID 26451910.