Pediatric Endocrine Tumors and Syndromes

Endocrine tumors occur rarely in childhood and their diagnosis, investigation and management requires a careful and coordinated approach from a wide range of experts. As most tumors occur much more commonly in adult practice, the multidisciplinary team should include experienced adult endocrinologists, surgeons and radiologists. Endocrine tumors in childhood are often one manifestation of a genetic endocrine tumor syndrome. Genetic testing should be considered in most affected children, as a syndromic diagnosis will inform tumor surveillance for the patient and genetic testing of first degree relatives. In this chapter the clinical presentation, careful evaluation and treatment of children presenting with these rare tumors will be discussed. Throughout the chapter, genetic syndromes known to be associated with each tumor will be highlighted.