Doege-Potter syndrome presenting as ‘end-stage renal disease-associated hypoglycaemia’: a primary presentation of retroperitoneal sarcoma

Abstract

A middle-aged woman with end-stage renal disease (ESRD) due to obstructive nephropathy presented to the hospital for an episode of unresponsiveness and hypoglycaemia. Initially, she was diagnosed with hypoglycaemia associated with ESRD and was discharged. However, she returned to the hospital after experiencing tonic–clonic seizures and recurrent hypoglycaemia. Her hypoglycaemia workup revealed an elevated insulin-like growth factor 2 (IGF2) to IGF1 ratio consistent with paraneoplastic IGF2 secretion. Subsequently, a CT abdomen revealed a retroperitoneal mass, found to be a retroperitoneal sarcoma. Her hypoglycaemia was treated with glucocorticoids and growth hormone. Surgical debulking of her tumour was attempted, but she expired due to postoperative haemorrhagic shock. Doege-Potter syndrome is a rare cause of hypoglycaemia which should be suspected in any new-onset, worsening, inexplicable or refractory hypoglycaemia, particularly in non-diabetic ESRD. Here we present a report of retroperitoneal sarcoma presenting with hypoglycaemia in a patient with ESRD without diabetes.