Pheochromocytoma and paraganglioma in the pediatric population

Rachel Kadakia*, Monica Bianco, Elizabeth Dabrowski, Donald Zimmerman

*Corresponding author for this work

Research output: Chapter in Book/Report/Conference proceedingChapter

1 Scopus citations

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.

Original languageEnglish (US)
Title of host publicationContemporary Endocrinology
PublisherHumana Press Inc.
Pages89-97
Number of pages9
DOIs
StatePublished - 2018

Publication series

NameContemporary Endocrinology
ISSN (Print)2523-3785
ISSN (Electronic)2523-3793

Keywords

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

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism
  • Endocrinology

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