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: Marisa Maibel Alface, Patricia Moniz, Susana Jesus, Cândida Fonseca
• Journal: BMJ case reports (BMJ Case Rep) Vol. 2015 (Aug 04 2015) ISSN: 1757-790X [Electronic] England
• PMID: 26243750 (Publication Type: Case Reports, Journal Article)
• Copyright: 2015 BMJ Publishing Group Ltd.

Chemical References

• Adrenergic Antagonists
• Metanephrine

Topics

• Adolescent
• Adrenal Gland Neoplasms (complications, diagnosis, metabolism, surgery)
• Adrenal Glands (pathology, surgery)
• Adrenergic Antagonists (therapeutic use)
• Blood Pressure
• Chromaffin Cells (pathology)
• Female
• Humans
• Hypertension (diagnosis, etiology, metabolism, surgery)
• Metanephrine (metabolism)
• Multiple Endocrine Neoplasia Type 2a
• Pheochromocytoma (complications, diagnosis, metabolism, surgery)