Multiple Endocrine Neoplasia Syndromes

  • Michael S. Racine
  • Beth A. Kurt
  • Pamela M. Thomas
Chapter

Abstract

The multiple endocrine neoplasia syndromes in children and adolescents present a challenging and varying group of familial tumor disorders, transmitted via autosomal dominant inheritance. Including MEN1, MEN2A and 2B, and familial medullary thyroid carcinoma, the MEN syndromes may affect children as young as 5 years, in MEN1, or as young as infants less than a year of age, as in MEN2-related medullary thyroid carcinoma (MTC). The MEN1 syndrome is composed primarily of pancreatic neuroendocrine tumors, parathyroid hyperplasia, and tumors of the anterior pituitary gland. The natural history of MEN1 in 160 children and adolescents under the age of 21 years has been elucidated in a large French series by the Groupe ďétude des Tumeurs Endocrines, shedding light on the varied combinations of presentations in young patients. The MEN2 syndromes are composed of MTC, pheochromocytoma, and more variably parathyroid hyperplasia, with a characteristic feature of MEN2B being mucosal neuromas. MEN2 is rarer than MEN1 and presents unique challenges in management, including the appropriate age for thyroidectomy and the evaluation and management of pheochromocytoma. DNA analysis has revolutionized the clinical management of MEN1 and MEN2. We discuss the diagnosis, management, and recommended screening strategies for this group of tumor syndromes, relative to the pediatric age group.

Keywords

Acromegaly Cushing’s disease Genetic Gastrinoma Hyperparathyroidism Hypoglycemia Insulinoma Medullary thyroid cancer Neoplasia Neuroendocrine Pancreatic neuroendocrine tumor Pheochromocytoma Pituitary adenoma Prolactinoma Tumor 

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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Michael S. Racine
    • 1
  • Beth A. Kurt
    • 2
  • Pamela M. Thomas
    • 3
  1. 1.Eugene EndocrinologyEugeneUSA
  2. 2.Pediatric Hematology and OncologySpectrum Health Helen DeVos Children’s HospitalGrand RapidsUSA
  3. 3.FayettevilleUSA

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