Recognition and management of phaeochromocytoma

Published:September 22, 2020DOI:


      Phaeochromocytomas and paragangliomas (PPGL) are catecholamine-secreting neuroendocrine tumours. These tumours may be identified incidentally, as part of a work-up for multiple endocrine neoplasia or following haemodynamic surges during unrelated procedures. Advances in preoperative management and improved management of intraoperative haemodynamic instability have significantly reduced surgical mortality from around 40% to less than 3%. Surgery is the definitive treatment in most cases and laparoscopic resection where possible is associated with improved outcomes. Anaesthetic management of PPGL cases represents a unique haemodynamic challenge both before, during and after tumour resection. In this article we describe the physiology of these tumours, their diagnosis, preoperative optimization methods, intraoperative anaesthetic management and management of postoperative complications.


      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to Anaesthesia & Intensive Care Medicine
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Lenders J.W.
        • Eisenhofer G.
        • Mannelli M.
        • Pacak K.
        Phaeochromocytoma. Lancet (London, England). 2005; 366: 665-675
        • Pillai S.
        • Gopalan V.
        • Smith R.A.
        • Lam A.K.
        Updates on the genetics and the clinical impacts on phaeochromocytoma and paraganglioma in the new era.
        Crit rev oncology/hematology. 2016; 100: 190-192
        • Salinas F.V.
        Contemporary perioperative and anesthetic management of pheochromocytoma and paraganglioma.
        Adv Anesth. 2016; 34: 181-196
        • Darr R.
        • Kuhn M.
        • Bode C.
        • et al.
        Accuracy of recommended sampling and assay methods for the determination of plasma-free and urinary fractionated metanephrines in the diagnosis of pheochromocytoma and paraganglioma: a systematic review.
        Endocrine. 2017; 56: 495-503
        • Eisenhofer G.
        • Goldstein D.S.
        • Walther M.M.
        • et al.
        Biochemical diagnosis of pheochromocytoma: how to distinguish true- from false-positive test results.
        J clin endocrinol metab. 2003; 88: 2656-2666
        • Lenders J.W.
        • Duh Q.Y.
        • Eisenhofer G.
        • et al.
        Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline.
        J clin endocrinol metab. 2014; 99: 1915-1942
        • Assalia A.
        • Gagner M.
        Laparoscopic adrenalectomy.
        Br J Surg. 2004; 91: 1259-1274
        • Connor D.
        • Boumphrey S.
        Preoperative care of phaeochromocytoma.
        BJA Education. 2016; 16: 153-158
        • Livingstone M.
        • Duttchen K.
        • Thompson J.
        • et al.
        Hemodynamic stability during pheochromocytoma resection: lessons learned over the last two decades.
        Ann Surg Oncol. 2013; 22: 4175-4180
        • Lord M.S.
        • Augoustides J.G.T.
        Perioperative management of pheochromocytoma: focus on magnesium, clevidipine, and vasopressin.
        J Cardiothorac Vasc Anesth. 2012; 26: 526-531