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Genetics of Endocrine and Neuroendocrine Neoplasias (PDQ®)

  • Updated: 07/17/2014

Table 2. Practice Guidelines for Surveillance of Multiple Endocrine Neoplasia Type 1 (MEN1)a

Biochemical Test or Procedure Condition Screened For Age Screening Initiated (y)  Frequency  
Serum prolactin and/or insulin-like growth factor 1Pituitary tumors5Every 1 y
Fasting total serum calcium and/or ionized calcium and PTHParathyroid tumors and PHPT8Every 1 y
Fasting serum gastrinDuodenopancreatic gastrinoma20Every 1 y
Chromogranin A, pancreatic polypeptide, glucagon, and vasointestinal polypeptidePancreatic NETs<10Every 1 y
Fasting glucose and insulinInsulinoma5Every 1 y
Brain MRIPituitary tumors5Every 3–5 y based on biochemical results
Abdominal CT or MRIb [4]Pancreatic NETs20Every 3–5 y based on biochemical results
Abdominal CT, MRI, or endoscopic USb [37]Pancreatic NETs<10Every 1 y

CT = computed tomography; MRI = magnetic resonance imaging; NETs = neuroendocrine tumors; PHPT = primary hyperparathyroidism; PTH = parathyroid hormone; US = ultrasound.
aAdapted from Brandi et al. [4] and Thakker et al. [37].
bThe recommendations for abdominal imaging differ between two published guidelines for the diagnosis and management of MEN1.[4,37] There is weak evidence at this time to support annual imaging before age 10 years. Imaging before age 10 years does identify disease in a high proportion of patients, but it is not clear whether this impacts prognosis.[20,57]

References

  1. Brandi ML, Gagel RF, Angeli A, et al.: Guidelines for diagnosis and therapy of MEN type 1 and type 2. J Clin Endocrinol Metab 86 (12): 5658-71, 2001.  [PUBMED Abstract]

  2. Thomas-Marques L, Murat A, Delemer B, et al.: Prospective endoscopic ultrasonographic evaluation of the frequency of nonfunctioning pancreaticoduodenal endocrine tumors in patients with multiple endocrine neoplasia type 1. Am J Gastroenterol 101 (2): 266-73, 2006.  [PUBMED Abstract]

  3. Thakker RV, Newey PJ, Walls GV, et al.: Clinical practice guidelines for multiple endocrine neoplasia type 1 (MEN1). J Clin Endocrinol Metab 97 (9): 2990-3011, 2012.  [PUBMED Abstract]

  4. Langer P, Kann PH, Fendrich V, et al.: Prospective evaluation of imaging procedures for the detection of pancreaticoduodenal endocrine tumors in patients with multiple endocrine neoplasia type 1. World J Surg 28 (12): 1317-22, 2004.  [PUBMED Abstract]