BACKGROUND: The purpose of this study was to analyze the effects of changes in the diagnosis and treatment of pheochromocytoma in a pediatric population. METHODS: We reviewed the medical records of all children who had resection of pheochromocytoma or paraganglioma at a major children's hospital since 1968. RESULTS: Fifteen children underwent surgery at 11.9 +/- 4.2 years of age. Presenting symptoms included headache, hypertension, and sweating. Three children had a mutation of the succinate dehydrogenase enzyme, and 1 child had nonsyndromic, familial pheochromocytoma. The most sensitive diagnostic modalities included 24-hour urinary and plasma norepinephrine and 24-hour urinary total metanephrines, magnetic resonance imaging, and 123I-meta-iodobenzylguanidine scintigraphy. Laparoscopic cortical-sparing adrenalectomy was performed in 3 patients with von Hippel-Lindau disease. Compared with those with open procedures (n = 7), patients who had laparoscopic resection (n = 5) had a statistically shorter hospital length of stay, and time to eating ambulation. CONCLUSIONS: The addition of 123I-meta-iodobenzylguanidine scanning, genetic testing, and laparoscopic surgery has changed the diagnosis and treatment of pheochromocytoma in children. Laparoscopic cortical-sparing adrenalectomy can be accomplished safely and is the preferred treatment for children at risk for multifocal disease.
BACKGROUND: The purpose of this study was to analyze the effects of changes in the diagnosis and treatment of pheochromocytoma in a pediatric population. METHODS: We reviewed the medical records of all children who had resection of pheochromocytoma or paraganglioma at a major children's hospital since 1968. RESULTS: Fifteen children underwent surgery at 11.9 +/- 4.2 years of age. Presenting symptoms included headache, hypertension, and sweating. Three children had a mutation of the succinate dehydrogenase enzyme, and 1 child had nonsyndromic, familial pheochromocytoma. The most sensitive diagnostic modalities included 24-hour urinary and plasma norepinephrine and 24-hour urinary total metanephrines, magnetic resonance imaging, and 123I-meta-iodobenzylguanidine scintigraphy. Laparoscopic cortical-sparing adrenalectomy was performed in 3 patients with von Hippel-Lindau disease. Compared with those with open procedures (n = 7), patients who had laparoscopic resection (n = 5) had a statistically shorter hospital length of stay, and time to eating ambulation. CONCLUSIONS: The addition of 123I-meta-iodobenzylguanidine scanning, genetic testing, and laparoscopic surgery has changed the diagnosis and treatment of pheochromocytoma in children. Laparoscopic cortical-sparing adrenalectomy can be accomplished safely and is the preferred treatment for children at risk for multifocal disease.
Authors: Herbert Chen; Rebecca S Sippel; M Sue O'Dorisio; Aaron I Vinik; Ricardo V Lloyd; Karel Pacak Journal: Pancreas Date: 2010-08 Impact factor: 3.327
Authors: Martin K Walz; Laura D Iova; Judith Deimel; Hartmut P H Neumann; Birke Bausch; Stefan Zschiedrich; Harald Groeben; Pier F Alesina Journal: World J Surg Date: 2018-04 Impact factor: 3.352