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Phaeochromocytoma and thrombotic microangiopathy: favourable outcome despite advanced renal failure
  1. Heiko Schweizer1,
  2. Joachim Boehm2,
  3. Jan Thorsten Winterer3,
  4. Damian Wild4,
  5. Hartmut P H Neumann1,
  6. Thorsten Wiech2,
  7. Mike Stubanus1,
  8. E Wolfgang Kuehn1
  1. 1Renal Division, University Hospital Freiburg, Freiburg, Germany
  2. 2Institute of Pathology, University Hospital Freiburg, Freiburg, Germany
  3. 3Department of Radiology, University Hospital Freiburg, Freiburg, Germany
  4. 4Department of Nuclear Medicine, University Hospital Freiburg, Freiburg, Germany
  1. Correspondence to Dr Joachim Böhm, University Hospital Freiburg, Institute of Pathology, Postfach 214, Freiburg 79002, Germany; joachim.boehm{at}

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Phaeochromocytomas are rare, mostly benign neuroendocrine tumours of the medulla of the adrenal glands that present as sporadic tumours or as manifestations of hereditary syndromes such as von Hippel–Lindau disease.1 Symptoms are mostly unspecific; occasionally the triad of headache, sweating and palpitations2 is found. Conversely, phaeochromocytomas can present acutely with malignant hypertension and varying clinical features ranging from rhabdomyolysis and myoglobinuric renal failure to haemorrhagic necrosis, pulmonary oedema3 and cardiogenic shock.4 Phaeochromocytomas with malignant hypertension frequently show a fatal clinical course, particularly when microangiopathic haemolytic anaemia is present.

A 28-year-old Caucasian woman presented with somnolence, diffuse abdominal pain, emesis, epistaxis, petaechiae and oliguria. She reported weight loss of 13 kg within a few weeks, palpitations, tremor, sweating and blurry vision. Her past medical history was unremarkable; she denied any history of hypertension. Her only medication were contraceptive pills. She had no relevant family history. Physical examination showed a somnolent patient with a blood pressure of 230/140 mmHg and a heart rate of 100 bpm. Her extremities showed extensive petechiae. The upper abdomen was tender without rigidity. The neurological examination was without focal deficit. Funduscopy showed cotton wool spots and striate haemorrhages but no papillary oedema. She had anaemia (haemoglobin 8.3 g/dl) and thrombocytopenia (76 000/mm3). Acute renal failure was present (creatinine 6.0 mg/dl). Microscopic examination of the urine revealed red cell casts. The urine protein:creatinine ratio was 8.3. Doppler …

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  • Competing interests None.

  • Patient consent Obtained.

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