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ADPKD Phaeochromocytoma Coincidental No Association — ESENeph MCQ

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HardPolycystic Kidney DiseaseADPKD Phaeochromocytoma Coincidental No AssociationESENeph

A patient with ADPKD has episodic headache, sweating, palpitations, markedly elevated metanephrines and an adrenal mass. How should the findings be interpreted?

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Correct answer: ADiagnose and manage phaeochromocytoma independently of the coexisting ADPKD

The best answer is “Diagnose and manage phaeochromocytoma independently of the coexisting ADPKD”. ADPKD does not explain a biochemically confirmed catecholamine-secreting adrenal tumour. Alpha blockade and preoperative preparation precede definitive surgery. “Attribute hypertension to ADPKD after excluding the functional adrenal lesion” is less appropriate because the biochemical and adrenal findings identify catecholamine excess “Characterize the adrenal lesion before deciding whether it is cystic” is less appropriate because adrenal phaeochromocytoma requires a separate pathway “Assess for MEN2 when the endocrine phenotype or family history supports it” is less appropriate because MEN2 requires its own endocrine and genetic phenotype “Establish alpha blockade before adding beta blockade when clinically required” is less appropriate because unopposed alpha stimulation can precipitate crisis

Reference: NHS guidance on phaeochromocytoma: https://www.nhs.uk/conditions/phaeochromocytoma/