Presenting signs and symptoms of pheochromocytoma in pediatric-aged patients

To describe the varied presenting signs and symptoms in pediatric patients with pheochromocytoma, a retrospective chart review of the presenting signs and symptoms and subsequent clinical course of patients who presented to the Pediatric ICU following surgical excision of a pheochromocytoma was undertaken. The cohort of 7 patients (5 boys, 2 girls) ranged in age from 4 to 16 years. Two patients were hypertensive at initial presentation, and the other 5 developed hypertension after their initial presentation. The initial presenting signs and symptoms were related to the central nervous system (CNS) in 6 of the patients (5 with an acute alteration in mental status and 1 with visual disturbances). Two patients presented with congestive heart failure. Other signs and symptoms at the time of initial presentation included sweating, headache, weight loss, heat intolerance, increased thirst and urination, a decline in school activity, and red/puffy hands and feet. The time from the initial presentation until the diagnosis was confirmed was 5 months or more in 4 of the 7 patients. The diagnosis was confirmed by demonstration of elevated urinary catecholamines in all 7 patients, although 2 patients had initial negative urinary levels.