Monitoring
New tumor monitoring is required for all patients with diagnosed or suspected multiple endocrine neoplasia (MEN). Lifelong monitoring is also important, even for those who have undergone successful surgery. This is because underlying genetic disorders can trigger recurrence of neuroendocrine tumors as new clonal tumors.[37] Monitoring intervals is tumor specific and should follow published guidelines.
Monitoring of known MEN1 carriers begins in childhood with annual biochemical screening and imaging every 2 to 3 years. MEN1 has poor genotype-to-phenotype correlation, meaning the clinical picture of affected relatives cannot be used to predict clinical courses. This makes screening with potentially useful serum markers difficult and expensive. Patients with undetermined carrier status require screening for the 2 most common tumors (primary hyperparathyroidism and prolactinoma). This screening involves measurement of serum levels every few years to detect carriers at early stages of disease. Those who have symptoms consistent with excess hormone production or suspicious cutaneous lesions require more detailed testing. MEN1 gene mutation carriers require screening every 3 years for adenomas with pituitary MRI, every year for pancreatic masses and every 3 years for adrenal masses with abdominal CT or MRI, and every 1-2 years for thymic or bronchopulmonary masses with chest CT.[3]
Monitoring for pheochromocytoma and hyperparathyroidism in patients with MEN2 can be targeted to those with mutations that confer increased risk of these tumors.
Annual biochemical screening of high-risk patients is recommended from the time of thyroidectomy. Patients with low-risk mutations require monitoring less often, but a consensus regarding frequency has not yet been reached.[5]
Many patients with MEN1 and MEN2 will have recurrent primary hyperparathyroidism (depending on the kind of surgery undergone) and will require lifelong calcium monitoring.[3]
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