Phaeochromocytoma and endocrine hypertension — branching viva

Branching viva from the hypertensive adolescent with spells, through the metanephrines-first diagnostic rule, the alpha-before-beta preoperative trap and the hereditary genetics, to the hypokalaemic child with suppressed renin and aldosterone whose gene dictates the drug.

branching clinical structured oral2 min readVerification in progress

Prompt

You are the paediatric registrar in the outpatient clinic and on the ward. The consultant asks you to assess three children: a hypertensive adolescent with episodic headache, sweating and palpitations; an eight-year-old with an adrenal mass spotted on ultrasound; and a ten-year-old with hypertension, hypokalaemia and a suppressed renin and aldosterone. The examiner releases information in stages.

Station opening

The consultant presents a 14-year-old boy with sustained hypertension and episodes of severe headache, sweating and palpitations lasting minutes, with pallor. Between episodes he feels well. The examiner asks: "What is the most likely diagnosis, and what single biochemical test would you order first?" [4]

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You have read the opening of this viva. The complete unit — every section and its primary-source references — is part of the Paediatrics Fellowship fellowship atlas.

References9Show ledger

[1]Lenders JW; Duh QY; Eisenhofer G; et al Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline. J Clin Endocrinol Metab, 2014.PMID 24893135
[2]Casey RT; Hendriks E; Deal C; et al International consensus statement on the diagnosis and management of phaeochromocytoma and paraganglioma in children and adolescents. Nat Rev Endocrinol, 2024.PMID 39147856
[3]Lenders JW; Pacak K; Walther MM; et al Biochemical diagnosis of pheochromocytoma: which test is best? JAMA, 2002.PMID 11903030
[4]Barontini M; Levin G; Sanso G Characteristics of pheochromocytoma in a 4- to 20-year-old population. Ann N Y Acad Sci, 2006.PMID 17102069
[5]Havekes B; Romijn JA; Eisenhofer G; et al Update on pediatric pheochromocytoma. Pediatr Nephrol, 2009.PMID 18566838
[7]Muth A; Crona J; Gimm O; et al Genetic testing and surveillance guidelines in hereditary pheochromocytoma and paraganglioma. J Intern Med, 2019.PMID 30536464
[8]Castinetti F; Waguespack SG; Machens A; et al Natural history, treatment, and long-term follow up of patients with multiple endocrine neoplasia type 2B: an international, multicentre, retrospective study. Lancet Diabetes Endocrinol, 2019.PMID 30660595
[11]New MI; Geller DS; Fallo F; et al Monogenic low renin hypertension. Trends Endocrinol Metab, 2005.PMID 15808805
[12]Garovic VD; Hilliard AA; Turner ST Monogenic forms of low-renin hypertension. Nat Clin Pract Nephrol, 2006.PMID 17066054