Multiple endocrine neoplasia type 1 screening

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ammu Susheela, M.D. [2]

Overview

According to the National Caner Institute, screening for multiple endocrine neoplasia type 1 by imaging studies such as brain MRI, abdominal CT and abdominal MRI is recommended every 3-5 year among patients with pituitary tumors and pancreatic neuroendocrine tumors respectively. Biochemical tests such as serum prolactin, insulin-like growth factor 1, fasting total serum calcium, ionized calcium, parathyroid hormone, fasting serum gastrin, chromogranin A, pancreatic polypeptide, glucagon and vaso-intestinal polypeptide are recommended every year among patients with pituitary tumors, pancreatic neuroendocrine tumors and primary hyperparathyroidism.

Screening

Screening and surveillance for multiple endocrine neoplasia type 1 may employ a combination of biochemical tests and imaging. Available recommendations are summarized in the following table. [1][2][3][4][5]

Biochemical Test or Procedure Condition Screened For Age Screening Initiated (y) Frequency
Serum prolactin and/or insulin-like growth factor 1 Pituitary tumors 5 Every 1 year
Fasting total serum calcium and/or ionized calcium and parathyroid hormone Parathyroidtumors and primary hyperparathyroidism 8 Every 1 year
Fasting serum gastrin Duodenopancreatic gastrinoma 20 Every 1 year
Chromogranin A, pancreatic polypeptide, glucagon, and vasointestinal polypeptide Pancreatic neuroendocrine tumors <10years Every 1 year
Fasting glucose and insulin Insulinoma 5 Every 1 year
Brain MRI Pituitary tumors 5 Every 3–5 years based on biochemical results
Abdominal CT or MRI Pancreatic neuroendocrine tumors 20 Every 3–5 years based on biochemical results
Abdominal CT, MRI, or endoscopic US Pancreatic neuroendocrine tumors <10 Every 1 year

References

  1. Skogseid B, Eriksson B, Lundqvist G, Lörelius LE, Rastad J, Wide L; et al. (1991). "Multiple endocrine neoplasia type 1: a 10-year prospective screening study in four kindreds". J Clin Endocrinol Metab. 73 (2): 281–7. doi:10.1210/jcem-73-2-281. PMID 1677362.
  2. Stock JL, Warth MR, Teh BT, Coderre JA, Overdorf JH, Baumann G; et al. (1997). "A kindred with a variant of multiple endocrine neoplasia type 1 demonstrating frequent expression of pituitary tumors but not linked to the multiple endocrine neoplasia type 1 locus at chromosome region 11q13". J Clin Endocrinol Metab. 82 (2): 486–92. doi:10.1210/jcem.82.2.3730. PMID 9024241.
  3. Baudin E, Bidart JM, Rougier P, Lazar V, Ruffié P, Ropers J; et al. (1999). "Screening for multiple endocrine neoplasia type 1 and hormonal production in apparently sporadic neuroendocrine tumors". J Clin Endocrinol Metab. 84 (1): 69–75. doi:10.1210/jcem.84.1.5445. PMID 9920064.
  4. Newey PJ, Jeyabalan J, Walls GV, Christie PT, Gleeson FV, Gould S; et al. (2009). "Asymptomatic children with multiple endocrine neoplasia type 1 mutations may harbor nonfunctioning pancreatic neuroendocrine tumors". J Clin Endocrinol Metab. 94 (10): 3640–6. doi:10.1210/jc.2009-0564. PMID 19622622.
  5. Falchetti A (2010). "Genetic screening for multiple endocrine neoplasia syndrome type 1 (MEN-1): when and how". F1000 Med Rep. 2. doi:10.3410/M2-14. PMC 2948394. PMID 20948872.

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