Endocrine Abstracts

Hypoglycaemia is a rare presenting feature of ACC. We present two patients at our centre presenting with hypoglycaemia subsequently diagnosed with advanced ACC. Both patients were refractory to treatment and subsequently succumbed to their disease. Non islet cell tumour hypoglycaemia (NICTH) in ACC is a rare paraneoplastic phenomenon caused by tumour overproduction of IGF-2 and its precursor (“big-IGF-2”). This results in activation of insulin receptors and subsequent hypoglycaemia. A 59-year-old women with a short history of weight loss and hirsutism presented with malignant hypertension and hypoglycaemia. Imaging showed a 20 cm left suprarenal mass with liver metastases. Biochemistry and subsequent biopsy confirmed a diagnosis of ACC not amenable to surgery. Investigation of hypoglycaemia found low insulin, low beta-hydroxybutyrate, normal range c-peptide and cortisol. Both IGF-2 and IGF-2:IGF-1 were elevated diagnostic of NICTH. Initial treatment of hypoglycaemia was with dietary changes and glucagon, however hypoglycaemia remained refractory to treatment, and she was admitted for a continuous infusion of 10% dextrose and prednisolone. She showed no improvement with octreotide, or dexamethasone and required ongoing dextrose infusion. She died while awaiting a trial of pasireotide. A 24-year-old man presented following a collapse at a music festival and was admitted with a low GCS and refractory hypoglycaemia requiring a 10% dextrose infusion and NG feed. Investigations of hypoglycaemia showed low insulin, low c-peptide and low beta-hydroxybutrate and a normal cortisol. Subsequent IGF-2:IGF-1 found to be elevated in keeping with NICTH. CT showed a large adrenal mass. Biochemistry confirmed ACC and he underwent surgical debulking and subsequent chemotherapy treatment with improvement in hypoglycaemia. Unfortunately, hypoglycaemia recurred with tumour progression requiring inpatient treatment with glucagon and dextrose infusions, GH treatment and NG feed. Patient deteriorated and died in hospital. These cases highlight a rare cause of hypoglycaemia and the difficulties in managing NICTH in advanced ACC.