Poster Presentation ESA-SRB-ANZBMS 2024 in conjunction with ENSA

A (phaeochromocytoma) crisis averted  (#625)

Peter Pham 1 , Elizabeth Wootton 1 2 , Maree Mungomery 1 , Kelly Stanton 1 2
  1. Royal Brisbane and Women's Hospital, Queensland Health, Brisbane, QLD, Australia
  2. University of Queensland, Brisbane, QLD, Australia

Case:A 43-year-old nurse in Vanuatu developed facial flushing, tremor, headache, and flank pain after taking oral glucocorticoids for trochanteric bursitis. Her previous medical history was unremarkable, and she had no family history of endocrinopathies. She was hypertensive and tachycardiac, and quickly progressed to respiratory distress – becoming tachypnoeic and hypoxic. Laboratory studies were significant for hyperglycaemia (8.9 mmol/L), acute renal impairment (creatinine 209 umol/L, urea 12.4 mmol/L), and leucocytosis (21.7 x 109/L). A chest X-ray revealed a heterogenous opacity in the right lower to upper zone. She was febrile and treated for severe community-acquired pneumonia causing septic shock with multi-organ failure, requiring intubation, inotropic support, and evacuation to ICU in Australia.

While the patient’s condition stabilised, she developed a type two myocardial infarction, and was commenced on Metoprolol for non-sustained ventricular tachycardia on telemetry. A CT pulmonary angiogram was negative for a pulmonary embolus; but unexpectedly, revealed an abdominal soft tissue lesion. This correlated to a 4.6 cm left adrenal mass with an average attenuation of 38 HU on an abdominal CT. Plasma normetanephrine (15,000 pmol/L, normal <1300 pmol/L) and metadrenaline (7,630 pmol/L, <540 pmol/L) were elevated.

Following adequate alpha- and beta-adrenergic blockade, she underwent an uncomplicated adrenalectomy, with histology confirming a phaeochromocytoma with intact SDHA/B immunochemistry. No germline variant was identified on genetic testing.

Only after treatment was it elucidated that the patient had experienced paroxysmal symptoms of catecholamine excess for twelve months prior to this presentation.

Discussion:Phaeochromocytoma crisis is a rare and life-threatening presentation of phaeochromocytomas1. It is characterised by haemodynamic instability and end-organ dysfunction, occurring in up to 20% of phaeochromocytoma cases2. Given the heterogeneity of phaeochromocytomas, and potential fatality if missed, clinicians must maintain a high index of suspicion for the diagnosis, especially in cases of diagnostic uncertainty that fail to improve with initial treatment.

 

 

 

  1. 1. Materazzi G, Rossi L, Papini P. Acute adrenal conditions: pheochromocytoma emergencies. In: Coccolini F, Catena F, editors. Textbook of Emergency General Surgery. Cham: Springer, 2023; pp 935–948.
  2. 2. Amar L, Eisenhofer G. Diagnosing phaeochromocytoma/paraganglioma in a patient presenting with critical illness: biochemistry versus imaging. Clinical Endocrinology 2015; 83: 298–302.