"When normal isn`t normal" a case of a biochemically silent pheochromocytoma / Nathaniel M. Tabije.

Includes appendices and bibliographical references.

Abstract: Pheochromocytoma is a rare clinically important disease in children presenting with hypertension. The lack of specificity of its manifestations highlights the importance of clinical suspicion. If left untreated, the outcome can be fetal. The diagnosis is usually established with biochemical studies measuring the levels of catecholamines or metabolites in urine or plasma, followed by radiological or imaging studies for localization. Most often surgical removal of the tumor is the treatment of choice in which pre-operative, Intra-operative and post-operative management is important. I report a 12-year-old adolescent who presented with hypertension, severe headaches and BMI score below -2 SD for age. KUB ultrasound and CT Angiogram revealed a focal, well circumscribed, strongly enhancing solid mass in the right supraenal region, extraepatic, extrarenal measurine 2.8x2.3x2.3cm. Investigations for adrenal hormones, including 24-hour urinary catecholamines as well as vanillylmandelic acid excretion, and plasma metanephrineswere all in normal ranges. As the features of the tumor on imaging studies suggested the diagnosis of pheochromocytoma, Surgical resection was performed. Histopathological study confirmed the diagnosis of pheochromocytoma. At present time he receives regular follow-up at our outpatient clinic with a stable clinical condition. Since nonfunctional adrenal tumors larger than 3 cm have higher potential to become functional, removal of the pheochromocytoma, which is "biochemically silent" in this patient, may prevent him from potentiallylethal complications in the future. (Formos J EndocrinMetab 2009; 1:33-37).

Research - Department of Child Health