Multiple endocrine neoplasia type 1 secondary prevention

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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 Cancer Institute, surveillance 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 vasoactive intestinal polypeptide are recommended every year for patients with pituitary tumors, pancreatic neuroendocrine tumors, and primary hyperparathyroidism.

Secondary Prevention

Surveillance for multiple endocrine neoplasia type 1 may employ a combination of biochemical tests and imaging. Available recommendations are summarized in the following table.

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 year based on biochemical results
Abdominal CT or MRI	Pancreatic neuroendocrine tumors	20	Every 3–5 year based on biochemical results
Abdominal CT, MRI, or endoscopic US	Pancreatic neuroendocrine tumors	<10	Every 1 year