title: The utility of cortical-sparing adrenalectomy in pheochromocytomas associated with genetic syndromes.
abstract: Management of pediatric patients with pheochromocytomas associated with genetic syndromes, such as Von Hippel-Lindau (VHL) disease, is complex owing to the need for continuous surveillance, high rates of recurrence, multiple operations during childhood, and the possibility of lifelong exogenous steroid replacement. We report the successful treatment of two siblings with VHL who underwent a total of six cortical-sparing procedures without requiring steroid therapy.
authors.affiliation: Division of Pediatric Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX, USA.
authors.forename: Sara C
authors.initials: SC
authors.lastname: Fallon
authors.forename: Daniel
authors.initials: D
authors.lastname: Feig
authors.forename: Monica E
authors.initials: ME
authors.lastname: Lopez
authors.forename: Mary L
authors.initials: ML
authors.lastname: Brandt
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000209:etiology / Q000601:surgery*; D000313:Adrenal Medulla / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D010673:Pheochromocytoma / Q000209:etiology / Q000601:surgery*; D035781:Siblings; D006623:von Hippel-Lindau Disease / Q000150:complications*
keywords: Adrenal Tumors; Pediatrics; Pheochromocytoma; Von Hippel Lindau