title: Pheochromocytoma: clinical review based on a rare case in adolescence.
abstract: Pheochromocytomas are rare tumours originating in chromaffin cells, representing 0.1-1% of all secondary hypertension (HT) cases. The majority are benign and unilateral, characterised by the production of catecholamines and other neuropeptides. Mainly located in the adrenal gland, they are more frequent between the 3rd and 5th decades of life; however, 10-25% can be associated with genetic familial syndromes (multiple endocrine neoplasia type 2 (MEN 2), type 1 neurofibromatosis and Von-Hippel-Landau disease in younger ages. The authors present a rare case of secondary HT due to a pheochromocytoma in a 15-year-old patient, whose metanephrine assay confirmed the diagnosis, and abdominal ultrasound and CT localised the tumour in the adrenal gland. HT was controlled with α and β blockers, with posterior retroperitoneal laparoscopic surgical intervention and subsequent resolution of HT. Age and concomitant hyperparathyroidism compelled genetic testing for the exclusion of MEN 2, which was negative.
authors.affiliation: Serviço de Medicina III, Hospital São Francisco Xavier-Centro Hospitalar Lisboa Ocidental (CHLO)/NOVA Medical School, Faculdade de Ciências Médicas, Universidade Nova de Lisboa, Lisbon, Portugal.
authors.forename: Marisa Maibel
authors.initials: MM
authors.lastname: Alface
authors.affiliation: Serviço de Medicina III, Hospital São Francisco Xavier-Centro Hospitalar Lisboa Ocidental (CHLO)/NOVA Medical School, Faculdade de Ciências Médicas, Universidade Nova de Lisboa, Lisbon, Portugal.
authors.forename: Patricia
authors.initials: P
authors.lastname: Moniz
authors.affiliation: Serviço de Medicina III, Hospital São Francisco Xavier-Centro Hospitalar Lisboa Ocidental (CHLO)/NOVA Medical School, Faculdade de Ciências Médicas, Universidade Nova de Lisboa, Lisbon, Portugal.
authors.forename: Susana
authors.initials: S
authors.lastname: Jesus
authors.affiliation: Serviço de Medicina III, Hospital São Francisco Xavier-Centro Hospitalar Lisboa Ocidental (CHLO)/NOVA Medical School, Faculdade de Ciências Médicas, Universidade Nova de Lisboa, Lisbon, Portugal.
authors.forename: Cândida
authors.initials: C
authors.lastname: Fonseca
mesh_terms: D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000378:metabolism / Q000601:surgery; D000311:Adrenal Glands / Q000473:pathology* / Q000601:surgery; D018674:Adrenergic Antagonists / Q000627:therapeutic use; D001794:Blood Pressure; D019439:Chromaffin Cells / Q000473:pathology*; D005260:Female; D006801:Humans; D006973:Hypertension / Q000175:diagnosis* / Q000209:etiology / Q000378:metabolism / Q000601:surgery; D008676:Metanephrine / Q000378:metabolism*; D018813:Multiple Endocrine Neoplasia Type 2a; D010673:Pheochromocytoma / Q000150:complications / Q000175:diagnosis* / Q000378:metabolism / Q000601:surgery
chemical_list: D018674:Adrenergic Antagonists; D008676:Metanephrine