2008 Annual Meeting Abstracts
Surgical Management of Two Consecutive Pediatric Paragangliomas with Aggressive Phenotypes.
Christopher S. Muratore, MD, Abigail Martin, MD, Mimi S. Kim, MD, Daniel S. Kim, MD, Shamlal Mangray, MD, Jose B. Quintos, MD, Thomas F. Tracy, Jr., MD.
Hasbro Children's Hospital / Alpert Medical School of Brown University, Providence, RI, USA.
Objective and Background
Catecholamine-secreting paragangliomas are rare neoplasms in children. Unlike in adults, pediatric pheochromocytomas are more often bilateral, extra-adrenal, multi-centric and malignant. Because they are uncommon, management of pediatric pheochromocytomas has historically been extrapolated from adult experience. We report two complex cases of paraganglioma presenting with significant complications requiring aggressive, non-traditional management in a multidisciplinary fashion.
Tertiary care children’s hospital
Two girls ages 15 and 7 without family history of pheochromocytoma or MEN syndrome presented in malignant hypertensive crisis. This was complicated by bilateral ischemic stroke, congestive heart failure, and rhabdomyolysis-induced acute renal failure in one and respiratory failure secondary to congestive heart failure in the other. Both required prolonged ventilatory management and echocardiography-directed inotropic support in addition to blood pressure management. Despite maximal multiagent alpha-and beta-adrenergic blockade, both required tyrosine hydroxylase inhibition (Metyrosine). Total plasma metanephrines reached 44,140 pg/ml and 6,145pg/ml (normal 50-200), respectively. Computed tomography scans demonstrated an 8.2 cm diameter retroperitoneal mass between the aorta and inferior vena cava (organ of Zuckerkandl) and caval thrombosis in one and a 6.5 cm left aortic-pararenal mass in the other.
Physiologic and plasma metanephrine normalization was eventually achieved and surgical resection was uneventful. Genomic DNA sequence analysis of the adolescent revealed a heterozygous nucleotide change in one copy of the succinate dehydrogenase (SDH) gene (glu to translation stop). No genetic abnormality has been identified in the younger patient.
We report a novel SDH-b mutational etiology in one child and sporadic etiology in another, both with severe clinical phenotypes affecting multiple organ systems and requiring aggressive peri-operative management.