Pheochromocytoma and paraganglioma: an Endocrine Society clinical practice guideline
The Endocrine Society guideline on catecholamine-producing tumours: test with plasma or urine metanephrines, image with CT or MRI and functional scans, offer genetic testing to everyone, block the blood pressure before surgery, and follow patients for life.
Overview
Clinical practice guideline recommending plasma free or urinary fractionated metanephrines for biochemical diagnosis, CT as first imaging with MRI and functional imaging (MIBG, FDG or somatostatin receptor PET) for metastatic or hereditary disease, shared decision-making on genetic testing for all patients with a clinical algorithm to prioritise genes, preoperative alpha-adrenergic blockade, minimally invasive adrenalectomy where appropriate, personalised management of metastatic disease, and lifelong follow-up, particularly for those with germline mutations.
The diagnostic, surgical and follow-up rows on both pheochromocytoma and paraganglioma pages follow this guideline.
- Predates the FIRSTMAPPP, belzutifan and high-specific-activity MIBG trials for metastatic disease.
- Evidence for most recommendations is low quality by design of the rare disease.
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