Glucagonoma screening

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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 of multiple endocrine neoplasia type 1 associated glucagonoma improves morbidity and survival rates of patients. Biochemical screening depends on measuring gastrointestinal hormones: gastrin, insulin, glucagon, VIP, pancreatic polypeptide, chromogranin A, prolactin, and IGF-1 in all patients. 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 result patients should have a periodic clinical, biochemical, and radiological screening program.

Screening

• Screening of multiple endocrine neoplasia type 1 associated glucagonoma improves morbidity and survival rates of patients.
• The survival rate of early diagnosed and treated glucagonoma is 85% while this rate falls to 60% in patients with malignant disease.
• Criteria of screening:

• • A first-degree relative of family member with known MEN1 mutation
  • Asymptomatic first-degree relative
  • First-degree relative with familial MEN1, for example, 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)

• Patients should be screened as early as possible; before 5 yr of age for asymptomatic patients.
• 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

Biochemical screening depended on measuring:

• • Serum concentrations of calcium in all patients as the earliest manifestation for MEN1 is hyperparathyroidism.
  • Gastrointestinal hormones: gastrin, insulin, glucagon, pancreatic polypeptide, chromogranin A, prolactin, and IGF-1 in all patients

• Pancreatic involvement in asymptomatic patients has been detected by measuring fasting plasma concentrations of gastrin, pancreatic polypeptide, glucagon, and chromogranin A and by abdominal imaging.
• 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 patients until the eighth decade.

Radiological screening

• Radiological screening should include an MRI of the pancreas, adrenal glands, and pituitary and repeated every 2 years.

Genetic counselling

• All high-risk patients should be offered genetic counseling and MEN1 mutation testing. If the genetic tests result patients should have a periodic clinical, biochemical, and radiological screening program.
• This may include examination for mutations in genes associated with familial hyperparathyroidism including CDC73 associated with the HPT-JT and the calcium sensing receptor (CASR), or cyclin-dependent kinase 1B (CDKN1B) and AIP which are rarely identified in those with clinical MEN1.