title: SDHB-Associated Paraganglioma in a Pediatric Patient and Literature Review on Hereditary Pheochromocytoma-Paraganglioma Syndromes.
abstract: Pheochromocytoma and paraganglioma are rare in the pediatric population occurring in approximately 1 in 50,000 children. While some cases are sporadic, they have commonly been associated with syndromes such as von Hippel-Lindau, multiple endocrine neoplasia types IIa and IIb, neurofibromatosis type 1, and hereditary pheochromocytoma-paraganglioma syndromes. In children less than 18 years of age approximately 60% of pheochromocytomas and paragangliomas are associated with a germline mutation. We present an 11-year-old child with an abdominal paraganglioma related to a succinate dehydrogenase subunit B gene mutation whose father had a previously resected abdominal paraganglioma and was found to carry the same mutation. In addition, we review the etiology, genetics, diagnostic approach, and challenges of preoperative management of secretory pheochromocytomas and paragangliomas in children.
authors.affiliation: Department of Pediatrics, University of South Carolina School of Medicine, Columbia, SC 29203, USA.
authors.forename: Heather
authors.identifier: 0000-0002-9874-1776
authors.initials: H
authors.lastname: Choat
authors.affiliation: Department of Pediatrics, University of South Carolina School of Medicine, Columbia, SC 29203, USA.
authors.forename: Kerri
authors.identifier: 0000-0002-8238-4604
authors.initials: K
authors.lastname: Derrevere
authors.affiliation: Division of Pediatric Endocrinology, Department of Pediatrics, University of South Carolina School of Medicine, 9 Medical Park Drive, Suite 230A, Columbia, SC 29203, USA.
authors.forename: Lisa
authors.identifier: 0000-0001-9335-0656
authors.initials: L
authors.lastname: Knight
authors.affiliation: Division of Pediatric Endocrinology, Department of Pediatrics, University of South Carolina School of Medicine, 9 Medical Park Drive, Suite 230A, Columbia, SC 29203, USA.
authors.forename: Whitney
authors.initials: W
authors.lastname: Brown
authors.affiliation: Division of Pediatric Critical Care, Palmetto Health Children's Hospital, Columbia, SC 29203, USA.
authors.forename: Elizabeth H
authors.initials: EH
authors.lastname: Mack