Pediatric implications of multiple endocrine neoplasia

The association of endocrine tumors from several sites has been known for over 50 years but the familial aspects of these relationships have only been appreciated since 1954. The original term multiple familial endocrine adenomatosis (MEA) was changed to multiple endocrine neoplasia (MEN). This report describes two children aged 8 and 11 years, who are cousins with MEN IIa. A strong family history prompted investigation of these children. Pentagastrin stimulation resulted in elevated serum calcitonin levels and subsequent surgery. Unsuspected medullary thyroid carcinoma was found in each child. Proper screening of high-risk individuals should prevent this potentially lethal condition from becoming a major problem.