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Pheochromocytoma and Paraganglioma in the Pediatric Population

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Pheochromocytomas, Paragangliomas and Disorders of the Sympathoadrenal System

Part of the book series: Contemporary Endocrinology ((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.

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Authors and Affiliations

  1. Department of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, IL, USA

    Rachel Kadakia, Monica Bianco, Elizabeth Dabrowski & Donald Zimmerman

  2. Division of Endocrinology, Ann and Robert H. Lurie Children’s Hospital of Chicago, Chicago, IL, USA

    Rachel Kadakia, Monica Bianco, Elizabeth Dabrowski & Donald Zimmerman

Authors
  1. Rachel Kadakia
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  2. Monica Bianco
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  3. Elizabeth Dabrowski
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  4. Donald Zimmerman
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Corresponding author

Correspondence to Rachel Kadakia .

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Editors and Affiliations

  1. Northwestern University, Feinberg School of Medicine, Chicago, IL, USA

    Lewis Landsberg

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Kadakia, R., Bianco, M., Dabrowski, E., Zimmerman, D. (2018). Pheochromocytoma and Paraganglioma in the Pediatric Population. In: Landsberg, L. (eds) Pheochromocytomas, Paragangliomas and Disorders of the Sympathoadrenal System. Contemporary Endocrinology. Humana Press, Cham. https://doi.org/10.1007/978-3-319-77048-2_5

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  • DOI: https://doi.org/10.1007/978-3-319-77048-2_5

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  • Publisher Name: Humana Press, Cham

  • Print ISBN: 978-3-319-77047-5

  • Online ISBN: 978-3-319-77048-2

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