14. Endocrine Neoplasia

  1. Charles G. D. Brook MA, MD, FRCP, FRCPCH1 and
  2. Mehul T. Dattani MD, FRCP, FRCPCH, DCH2,3

Published Online: 23 FEB 2012

DOI: 10.1002/9781119968153.ch14

Handbook of Clinical Pediatric Endocrinology, Second Edition

Handbook of Clinical Pediatric Endocrinology, Second Edition

How to Cite

Brook, C. G. D. and Dattani, M. T. (2012) Endocrine Neoplasia, in Handbook of Clinical Pediatric Endocrinology, Second Edition, Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781119968153.ch14

Author Information

  1. 1

    University College London, London, UK

  2. 2

    Developmental Endocrinology Research Group, Clinical and Molecular Genetics Unit, UCL Institute of Child Health, UK

  3. 3

    Great Ormond Street Hospital for Children, London and University College London Hospitals, UK

Publication History

  1. Published Online: 23 FEB 2012
  2. Published Print: 13 APR 2012

ISBN Information

Print ISBN: 9780470657881

Online ISBN: 9781119968153

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Keywords:

  • adrenal neoplasia;
  • multiple endocrine neoplasia (MEN);
  • ovarian tumors;
  • tumors of the testes

Summary

This chapter describes the problem of endocrine neoplasia, which is rare in childhood. Its management is challenging and requires collaboration between the pediatric endocrinologist and oncologists, specialist surgeons, geneticists, clinical biochemists, pathologists, radiotherapists and anesthetists working as a team in collaboration with specialists in adult practice to facilitate seamless transition into adult medical services. Many children require lifelong endocrine therapies or surveillance for recurrent or associated disease. The rarity of these tumors is reflected in a lack of knowledge of the natural history of some of the conditions and a paucity of evidence informing management of advanced or relapsed disease. National and international collaborations seek to address these deficiencies by establishing disease registries and delivering rigorous clinical trials. Endocrine neoplasia often occurs in the context of genetic abnormalities, which may be associated with an increased lifetime risk of malignant disease in a number of endocrine or non-endocrine organs.