Table 1.

Surveillance for MEN1 mutation carriers

MEN1 manifestationScreen starting at ageClinical screeningAnnual biochemical testsImaging
Insulinoma5 yrsSyncope, light-headedness, documented hypoglycemiaFasting glucose and insulinNone
PitNET5 yrsaHeadaches, visual changes, galactorrhea, ⇑growthProlactin, IGF-1Brain MRI (q 3 yrs)
Parathyroid adenoma/1o HyperPTH8 yrsBack pain, bone pain, weakness, fatigue, psychiatric changes, kidney stones, nausea, vomiting, constipation. Multiple or pathologic fractures.CalciumbNone
Pancreatic NET10 yrsGenerally not identified symptomatically. VIPoma can cause profuse diarrhea. Glucagonoma associated with hyperglycemia, nausea, polyuria, thirst.(Chromogranin A, glucagon, proinsulin, pancreatic polypeptide, VIP)cAbdominal MRI (annually)
Adrenal adenoma10 yrsNoneNoneMRI (contemporaneous with pancreatic imaging)
Gastrointestinal, bronchial, and thymic NETs20 yrsFrequently asymptomatic, but h/o flushing, diarrhea, wheezing, edema or abdominal pain should arouse suspicionCT/MRI chest and abdomen (q 1–2 yrs)
Gastrinoma (duodenal and pancreatic)20 yrsAbdominal pain, gastric ulcers. Proton-pump inhibitor usage.Fasting gastrinNone
  • NOTE: Data from Thakker et al.; ref. 5).

  • Abbreviations: h/o, history of; HyperPTH, hyperparathyroidism; NET, neuroendocrine tumor; q, every; VIP, vasoactive intestinal polypeptide; yrs, years.

  • aMRI surveillance is to begin once patient is able to tolerate a nonsedated MRI. In the authors' experience, this is generally at about the age of 5 years but may be deferred on an individualized basis.

  • bHypercalcemia on screening should prompt assessment with contemporaneous serum calcium and intact parathyroid hormone (iPTH) to establish a diagnosis of PHPT.

  • cPancreatic tumors may be nonsecretory, therefore, the added sensitivity contributed by biochemical screening has not been demonstrated.