Thoracic pheochromocytoma revealed by ventricular tachycardia - Clinical case and review of the literature

A case of thoracic extra-adrenal pheochromocytoma was observed in a 13-year -old girl. The initial clinical symptoms consisted of her being unwell afte r effort over a period of 3 years. The diagnosis was suggested after a reco rded episode of paroxysmal ventricular tachycardia with concomitant hyperte nsion, which reproduced the characteristic symptoms. Biochemical analysis c onfirmed hypersecretion of catecholamines and morphological examinations sh owed the presence of an isolated left median thoracic tumor. One year after surgical excision, the patient remained completely asymptomatic, in spite of resuming intense sporting activity. The diagnosis of ectopic pheochromoc ytoma should be considered as a possible etiology of cardiac rhythm disturb ances or hypertension in children. Thorough biochemical and radiological ex amination including MIBG radioisotope scanning should be carried out system atically before surgical excision; pheochromocytomas in children are freque ntly extra-adrenal (30%), multifocal (50%) or associated with multiple endo crine neoplasms (NEM).