Phaeochromocytoma and thrombotic microangiopathy: favourable outcome despite advanced renal failure

摘要

Phaeochromocytomas are rare, mostly benign neuroendocrine tumours of thernmedulla of the adrenal glands that present as sporadic tumours or as manifestations of hereditary syndromes such as von Hippel-Lindau disease. Symptoms are mostly unspecific; occasionally the triad of headache, sweating and palpitations 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 oedema and cardiogenic shock. Phaeochromocytomas with malignant hypertension frequently show a fatal clinical course, particularly when microangiopathic haemolytic anaemia is present.