Pheochromocytoma and Paraganglioma in the Pediatric Population

  • Rachel Kadakia
  • Monica Bianco
  • Elizabeth Dabrowski
  • Donald Zimmerman
Chapter
Part of the Contemporary Endocrinology book series (COE)

Abstract

Pheochromocytomas and paragangliomas are rare tumors in the pediatric population that arise from neural crest cells. Prompt recognition and treatment are necessary to limit morbidity. Compared to adults, pheochromocytoma and paraganglioma in children are more likely to be associated with an underlying genetic syndrome, and genetic testing should be pursued. These tumors can produce catecholamines and present with symptoms such as sweating, nausea, emesis, hypertension, or diarrhea, or they can be nonfunctional and present with symptoms related to mass effect. Evaluation is initiated with measurement of urine and plasma metanephrines and diagnosis confirmed with magnetic resonance imaging. Definitive treatment occurs via surgical excision. A multidisciplinary team specifically trained in the management of pediatric PHEO and PGL is necessary for optimal patient care.

Keywords

Pheochromocytoma Paraganglioma Catecholamine Multiple endocrine neoplasia Familial PGL syndrome Von Hippel-Lindau syndrome 

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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Rachel Kadakia
    • 1
    • 2
  • Monica Bianco
    • 1
    • 2
  • Elizabeth Dabrowski
    • 1
    • 2
  • Donald Zimmerman
    • 1
    • 2
  1. 1.Department of PediatricsNorthwestern University Feinberg School of MedicineChicagoUSA
  2. 2.Division of EndocrinologyAnn and Robert H. Lurie Children’s Hospital of ChicagoChicagoUSA

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