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Pheochromocytoma crisis in a patient with newly diagnosed neurofibromatosis type 1
  1. Hisashi Murakami,
  2. Tomohiro Sonoo,
  3. Hideki Hashimoto and
  4. Kensuke Nakamura
  1. Emergency and Critical Care Medicine, Hitachi General Hospital, Hitachi, Ibaraki, Japan
  1. Correspondence to Dr Kensuke Nakamura; mamashockpapashock{at}


Pheochromocytoma occasionally engenders catecholamine-induced hypertension crisis. Pheochromocytoma is clinically identified in 0.1%–5.7% of patients with neurofibromatosis type 1 (NF1), which is 10 times more frequently than in healthy individuals. This report describes a case of newly diagnosed NF1 presenting with pheochromocytoma crisis, with severe electrolyte depletion and deteriorating recurrent ventricular tachycardia storm. Characteristic skin lesions such as café-au-lait macules and neurofibromas contributed to the diagnosis of NF1 and pheochromocytoma. No recurrence of electrolyte depletion was found after the adrenalectomy. Primary care physicians must distinguish the characteristic skin lesions of NF1, such as café-au-lait macules and neurofibromas and recognise the risk for pheochromocytoma.

  • adult intensive care
  • adrenal disorders

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  • Contributors HM: conception and interpretation, drafting the manuscript. TS: contribution to the manuscript. HH: contribution to the manuscript. KN: revisions of the manuscript and supervised. All authors have read and approved the manuscript.

  • 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.