TY - CHAP
T1 - Pheochromocytoma and paraganglioma in the pediatric population
AU - Kadakia, Rachel
AU - Bianco, Monica
AU - Dabrowski, Elizabeth
AU - Zimmerman, Donald
N1 - Publisher Copyright:
© 2018, Springer International Publishing AG, part of Springer Nature.
PY - 2018
Y1 - 2018
N2 - 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.
AB - 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.
KW - Catecholamine
KW - Familial PGL syndrome
KW - Multiple endocrine neoplasia
KW - Paraganglioma
KW - Pheochromocytoma
KW - Von Hippel-Lindau syndrome
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U2 - 10.1007/978-3-319-77048-2_5
DO - 10.1007/978-3-319-77048-2_5
M3 - Chapter
AN - SCOPUS:85064854794
T3 - Contemporary Endocrinology
SP - 89
EP - 97
BT - Contemporary Endocrinology
PB - Humana Press Inc.
ER -
