title: Thoracic pheochromocytoma revealed by ventricular tachycardia. Clinical case and review of the literature.
abstract: A case of thoracic extra-adrenal pheochromocytoma was observed in a 13-year-old girl. The initial clinical symptoms consisted of her being unwell after effort over a period of 3 years. The diagnosis was suggested after a recorded episode of paroxysmal ventricular tachycardia with concomitant hypertension, which reproduced the characteristic symptoms. Biochemical analysis confirmed hypersecretion of catecholamines and morphological examinations showed 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 pheochromocytoma should be considered as a possible etiology of cardiac rhythm disturbances or hypertension in children. Thorough biochemical and radiological examination including MIBG radioisotope scanning should be carried out systematically before surgical excision; pheochromocytomas in children are frequently extra-adrenal (30%), multifocal (50%) or associated with multiple endocrine neoplasms (NEM).
authors.affiliation: Department of Pediatric Surgery, Hôpital Robert Debré, Paris, France.
authors.forename: T
authors.initials: T
authors.lastname: Petit
authors.forename: P
authors.initials: P
authors.lastname: de Lagausie
authors.forename: J
authors.initials: J
authors.lastname: Maintenant
authors.forename: S
authors.initials: S
authors.lastname: Magnier
authors.forename: Y
authors.initials: Y
authors.lastname: Nivoche
authors.forename: Y
authors.initials: Y
authors.lastname: Aigrain
mesh_terms: D000293:Adolescent; D005260:Female; D006801:Humans; D008479:Mediastinal Neoplasms / Q000150:complications / Q000175:diagnosis*; D010673:Pheochromocytoma / Q000150:complications / Q000175:diagnosis*; D017180:Tachycardia, Ventricular / Q000209:etiology*