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Case report
Biochemistry may be misleading in metachronous MEN2A-associated phaeochromocytoma following unilateral total adrenalectomy
  1. Anthony-Joe Nassour1,2,
  2. Carolina Nylen3,
  3. Mark Sywak1 and
  4. Roderick Clifton-Bligh4
  1. 1Endocrine Surgery, Royal North Shore Hospital School, Saint Leonards, New South Wales, Australia
  2. 2General Surgery, Royal North Shore Hospital School, St Leonards, New South Wales, Australia
  3. 3Department of Breast and Endocrine Surgery, Karolinska University Hospital, Stockholm, Sweden
  4. 4Endocrinology, Royal North Shore Hospital School, Saint Leonards, New South Wales, Australia
  1. Correspondence to Dr Anthony-Joe Nassour; anthonyjnas{at}


A 63-year-old woman with multiple endocrine neoplasia type 2A (MEN2A) presented with recurrent spells of headaches, sweats and palpitations decades after right adrenalectomy for phaeochromocytoma, and total thyroidectomy for medullary thyroid cancer. She was hypertensive and in sinus rhythm. DOTA-TATE positron-emission tomography (PET) demonstrated a 12mm enhancing left adrenal incidentaloma. 24 hours urine catecholamines, and multiple plasma metanephrine and normetanephrine measurements were all within normal reference ranges. Based on her symptoms and imaging findings, left adrenalectomy was performed and found a 40 mm phaeochromocytoma. Her symptoms have since completely resolved and plasma metanephrine is now undetectable MEN2-associated phaeochromocytomas are often bilateral and may be metachronous. Patients at high risk of phaeochromocytoma who develop symptoms of catecholamine excess should be carefully evaluated even if plasma or urinary metanephrines are within the normal reference range. Biochemical reference ranges for metanephrines need to be adjusted accordingly in patients who have had prior unilateral total adrenalectomy.

  • surgical diagnostic tests
  • endocrinology
  • adrenal disorders
  • endocrine cancer
  • general surgery

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  • Contributors All selected authors substantially contributed to the conception, literature review and revision of the final approved version of this case report. A-JN, primary author, conducting the literature search and review. Obtaining patient consent and maintaining follow-up. Write up and design of the first draft. CN contributed significantly to the revision and final draft of the case presentation and epidemiology component in the discussion section. MS, the primary surgeon, performing nephrectomy. Contributed significantly to the revision and final draft of the case report. Particularly to the treatment and discussion section. RC-B, endocrinologist, provided conception and learning topics for this case report. Contributed significantly to the final version of the entire case report.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.