Glucagonoma screening: Difference between revisions
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{{Glucagonoma}} | {{Glucagonoma}} | ||
{{CMG}}{{AE}}{{PSD}} | {{CMG}}; {{AE}} {{PSD}} {{MAD}} | ||
==Overview== | ==Overview== | ||
==Screening== | ==Screening== | ||
Screening of glucagonoma related to multiple endocrine neoplasia 1 improve morbidity and surviving rates. | |||
When to screen? | |||
Patients should be screened as early as possible; before 5 yr of age for asymptomatic individuals. | |||
Who to screen? | |||
* A first-degree relative of family member with known MEN1 mutation | |||
* Asymptomatic first-degree relative | |||
* First-degree relative with familial MEN1 (i.e. one MEN1-associated tumor) | |||
* Suspicious multiple parathyroid adenomas before the age of 40 yr; recurrent hyperparathyroidism; gastrinoma or multiple pancreatic NET at any age) or atypical for MEN1 (i.e. development of two nonclassical MEN1-associated tumors, e.g. parathyroid and adrenal tumor) | |||
How to screen? | |||
Biochemical screening for the development of MEN1 tumors in asymptomatic members of families with MEN1 is likely to be of benefit in as much as earlier diagnosis and treatment of these tumors may help reduce morbidity and mortality. | |||
Screening for MEN1 tumors is difficult because clinical and biochemical manifestations in members of any one family are not uniformly | |||
similar. | |||
Biochemical screening depended on measuring: | |||
* Serum concentrations of calcium | |||
* Gastrointestinal hormones: gastrin, insulin, glucagon, VIP, and pancreatic polypeptide chromogranin A, prolactin, and IGF-I in all individuals | |||
Pancreatic involvement in asymptomatic individuals has been detected by measuring fasting plasma concentrations of gastrin, pancreatic polypeptide, glucagon, and chromogranin A and by abdominal imaging. (4, 102) | |||
Screening should be done at least once annually and also have baseline pituitary and abdominal imaging which should then be repeated at 1- to 3-yr intervals and it should be repeated throughout life because the disease may not manifest in some individuals until the eighth decade. | |||
Radiological screening should include an MRI of the pancreas, adrenal glands, and pituitary and repeated every 2 years. | |||
All high-risk patients should be offered genetic counseling and MEN1 mutation testing. If the genetic tests reults patients should have a periodic clinical, biochemical, and radiological screening program. | |||
This may include examination for mutations in genes associated with familial parathyroid syndromes including CDC73 associated with the HPT-JT and the calcium sensing receptor (CASR) associated with FBHH; or cyclin-dependent kinase 1B (CDKN1B) and AIP which are rarely identified in those with clinical MEN1. | |||
==References== | ==References== | ||
{{reflist|2}} | {{reflist|2}} | ||
Revision as of 17:35, 4 August 2017
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Glucagonoma Microchapters |
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Case Studies |
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Glucagonoma screening On the Web |
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American Roentgen Ray Society Images of Glucagonoma screening |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Parminder Dhingra, M.D. [2] Mohammed Abdelwahed M.D[3]
Overview
Screening
Screening of glucagonoma related to multiple endocrine neoplasia 1 improve morbidity and surviving rates. When to screen? Patients should be screened as early as possible; before 5 yr of age for asymptomatic individuals. Who to screen?
- A first-degree relative of family member with known MEN1 mutation
- Asymptomatic first-degree relative
- First-degree relative with familial MEN1 (i.e. one MEN1-associated tumor)
- Suspicious multiple parathyroid adenomas before the age of 40 yr; recurrent hyperparathyroidism; gastrinoma or multiple pancreatic NET at any age) or atypical for MEN1 (i.e. development of two nonclassical MEN1-associated tumors, e.g. parathyroid and adrenal tumor)
How to screen? Biochemical screening for the development of MEN1 tumors in asymptomatic members of families with MEN1 is likely to be of benefit in as much as earlier diagnosis and treatment of these tumors may help reduce morbidity and mortality. Screening for MEN1 tumors is difficult because clinical and biochemical manifestations in members of any one family are not uniformly similar. Biochemical screening depended on measuring:
- Serum concentrations of calcium
- Gastrointestinal hormones: gastrin, insulin, glucagon, VIP, and pancreatic polypeptide chromogranin A, prolactin, and IGF-I in all individuals
Pancreatic involvement in asymptomatic individuals has been detected by measuring fasting plasma concentrations of gastrin, pancreatic polypeptide, glucagon, and chromogranin A and by abdominal imaging. (4, 102) Screening should be done at least once annually and also have baseline pituitary and abdominal imaging which should then be repeated at 1- to 3-yr intervals and it should be repeated throughout life because the disease may not manifest in some individuals until the eighth decade. Radiological screening should include an MRI of the pancreas, adrenal glands, and pituitary and repeated every 2 years. All high-risk patients should be offered genetic counseling and MEN1 mutation testing. If the genetic tests reults patients should have a periodic clinical, biochemical, and radiological screening program. This may include examination for mutations in genes associated with familial parathyroid syndromes including CDC73 associated with the HPT-JT and the calcium sensing receptor (CASR) associated with FBHH; or cyclin-dependent kinase 1B (CDKN1B) and AIP which are rarely identified in those with clinical MEN1.