Table of contents
Pheochromocytoma and paraganglioma
Background
Overview
Definition
Pheochromocytomas and paragangliomas are rare NETs that originate from chromaffin cells in the adrenal medulla or extra-adrenal paraganglia, respectively.
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Pathophysiology
The pathophysiology of these tumors involves the uncontrolled growth of chromaffin cells, which are responsible for the production of catecholamines. This overproduction can lead to a variety of symptoms and complications, including hypertensive crisis and CVD.
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Epidemiology
The overall incidence of pheochromocytoma or paraganglioma in Canada is estimated to be 0.66 cases per 100,000 people per year.
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Risk factors
Risk factors for pheochromocytoma and paraganglioma include certain genetic syndromes, such as MEN2, neurofibromatosis type 1, and von Hippel-Lindau syndrome. Additionally, mutations in the succinate dehydrogenase gene family have been found to predispose individuals to pheochromocytoma.
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Disease course
Clinically, these tumors often present with symptoms related to catecholamine overproduction. These can include hypertension, palpitations, sweating, panic attacks, and pallor. Symptoms can be paroxysmal or persistent and may be triggered by certain medications, changes in abdominal pressure, or even micturition.
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Prognosis and risk of recurrence
The prognosis of these tumors is generally favorable if diagnosed early and managed appropriately. However, if left untreated or if diagnosis is delayed, these tumors can metastasize and lead to severe complications.
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Guidelines
Key sources
The following summarized guidelines for the evaluation and management of pheochromocytoma and paraganglioma are prepared by our editorial team based on guidelines from the Canadian Urological Association (CUA 2023,2019), the American Association of Endocrine Surgeons (AAES 2022), the European Reference Network on Rare Adult Solid Cancers (EURACAN/ESMO 2020), the Canadian Hypertension Education Program (CHEP 2018,2016), the European Society of Endocrinology ...
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Screening and diagnosis
Classification and risk stratification
Diagnostic investigations
Clinical assessment: as per ESMO/EURACAN 2020 guidelines, obtain careful clinical assessment for signs of adrenal hormone excess in all patients with suspected pheochromocytoma.
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Metanephrine assays
Chromogranin A
Diagnostic imaging
Genetic testing
Diagnostic procedures
Perioperative care
Preoperative alpha-blockers: as per AAES 2022 guidelines, administer either selective or nonselective α-blockers, depending on the drug availability/cost, experience, and preference of the care team, to safely prepare patients for surgical resection of pheochromocytoma or paraganglioma. Recognize that selective α-blockers (doxazosin, prazosin, terazosin) are associated with more intraoperative hemodynamic instability while nonselective α-blockers (phenoxybenzamine) result in more postoperative hypotension, although there is no significant difference in morbidity or mortality between groups.
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Perioperative monitoring
Surgical interventions
Indications for adrenalectomy: as per CUA 2023 guidelines, perform adrenalectomy, with a minimally invasive technique when feasible, in patients with pheochromocytomas.
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Choice of surgical approach
Indications for debunking surgery
Specific circumstances
Follow-up and surveillance
Indications for tertiary care referral: as per ESMO/EURACAN 2020 guidelines, refer all patients with suspected and proven pheochromocytoma or paraganglioma to be discussed in a multidisciplinary expert team meeting, at least at the time of initial diagnosis and in case of progressive disease.
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Follow-up (laboratory)
Follow-up (imaging)
Follow-up (duration)