Paeds Cases · endocrinology-diabetes-and-growth
Phaeochromocytoma and endocrine hypertension — structured clinical encounter
Structured encounter testing the approach to a hypertensive adolescent with episodes of severe headache, sweating and palpitations: the metanephrines-first diagnostic rule, the imaging strategy, the alpha-before-beta preoperative preparation, the hereditary gene panel, and the family conversation about lifelong surveillance.
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RACP General PaediatricsRACP DCEMRCPCH ClinicalRCPSC Pediatrics
Prompt
A 13-year-old boy presents with a six-month history of episodes of severe throbbing headache, generalised sweating, palpitations and a feeling of dread lasting five to ten minutes, with pallor noted by his mother. Between episodes he feels well and plays sport. His blood pressure is 158/98, sustained on repeat measurement, with a heart rate of 94. You are the paediatric registrar working through the diagnosis, the confirmatory work-up, the preoperative preparation and the conversation with the family.
Station brief (candidate)
You have ten minutes to assess this boy, establish the diagnosis, outline the investigation and preoperative management, and address the family's questions. The examiner will prompt you at intervals. A catecholamine-secreting tumour is the leading diagnosis, and the family is frightened by the severity of the spells. [4]
References6ShowHide
- [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