Successful multidisciplinary treatment of Doege-Potter syndrome: hypoglycaemia caused by paraneoplastic IGF-2 production by a metastatic haemangiopericytoma

Abstract

Hypoglycaemia due to insulin-like growth factor (IGF)-2 secretion is a paraneoplastic complication of malignancy with significant morbidity that can often go unrecognised due to its uncommon presentation. We report on a case of a 51-year-old man with metastatic haemangiopericytoma presenting with refractory hypoglycaemia, requiring continuous dextrose 10% infusion while in hospital. IGF-2 levels were significantly elevated, in keeping with a rare entity associated with solitary fibrous tumours, known as Doege-Potter syndrome. The patient was managed using uncooked cornstarch in conjunction with debulking of the hepatic tumour burden with bland IR-guided transarterial embolisation, and eventual surgical resection to treat his non-islet cell tumour hypoglycaemia (NICTH). The case highlights this rare paraneoplastic phenomenon that should be included in the differential for hypoglycaemia, especially if a history of a solitary fibrous tumour is elicited. Our case is the first to document a successful approach to treating the hypoglycaemia using preoperative transarterial bland embolisation.