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INTRODUCTION

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Most endocrine tumors in children occur in the context of genetic conditions predisposing to multiple neoplasias. Therefore, it is essential that the evaluation and management of these patients involve experts in cancer genetics and include formal genetic counseling. If a family-specific mutation is found, the genetics consultant can offer mutation-specific predictive testing to relatives. A list of endocrine neoplasia syndromes and their genetic causes is provided in Table 530-1. Disease associations in the multiple endocrine neoplasia syndromes are shown in Table 530-2. In this chapter, we will discuss only the multiple endocrine neoplasia (MEN) conditions and the related Carney complex, all of which are autosomal dominant disorders in which specific endocrine glands and other organs develop hyperplasia or neoplasia. Although more often seen in adults, these conditions may present in childhood. Pheochromocytoma is also discussed as an endocrine tumor common in a number of endocrine neoplasia syndromes. It is extremely important to diagnose these genetic conditions during childhood in order to provide periodic screening for the development of neoplasia and to offer timely prophylactic surgery.

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Table Graphic Jump Location
TABLE 530-1ENDOCRINE AND OTHER TUMORS IN CHILDHOOD AND YOUNG ADULTHOOD AND THEIR GENETIC CAUSES
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Table Graphic Jump Location
TABLE 530-2DISEASE ASSOCIATIONS IN THE MULTIPLE ENDOCRINE NEOPLASIA SYNDROMES

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