Endocrine Abstracts

Introduction: Spontaneous hypoglycaemia is very rarely related to non-islet cell tumours. Most of them are due to mesenchymal tumours including haemangiopericytoma (HAP). It has been reported that, in patients with NICTH who have inoperable tumours, high doses of glucocorticoid or recombinant human growth hormone may be effective in treating hypoglycaemia. We present, with relevant imaging, a rare case of metastatic HAP causing an increased level of insulin like growth factor (IGF) 2 causing spontaneous hypoglycaemia.

Case report: A 62-year-old lady presented to the accident and emergency department with recurrent spontaneous hypoglycaemia (blood glucose 2.7 mmol/l). Her past medical history included recurrent para-sagittal HAP treated with three separate craniotomies and radiotherapy.

Investigations: On 72 h fasting, hypoglycaemia (1.9 mmol/l) was confirmed with appropriately suppressed insulin (<21 pmol/l) and C-peptide (<169 pmol/l) levels, ruling out an insulinoma. Short synacthen test showed an adequate cortisone response (peak 1120 mmol/l). Her IGF1 level was reduced (<3) with an increased IGF2 level (70.2 nmol/l) and IGF2: IGF1 ratio (17.6; reference value <10).

Computed tomography of chest and abdomen showed multiple liver and pulmonary metastases. Liver biopsy revealed a poorly differentiated diffuse malignant tumour with sarcomatoid appearance with a low mitotic rate. Cluster of differentiation 99 was strongly expressed. It was negative for cytokeratin, lymphoid antigens, melanoma antigens and neuroendocrine antigens. The immunocytochemistry result was not definitive but consistent with monophasic sarcoma related to previous HAP.

Treatment: She was commenced on prednisolone (30 mg once a day) with consequent improvement in hypoglycaemic episodes. Liver and hepatic nodules reduced in size after chemotherapy with doxorubicin.

Conclusion: Our case report serves to highlight a very rare cause of IGF2 producing tumour resulting in non- islet cell tumour hypoglycaemia. In addition, it also demonstrates the role of high dose steroids in this case, as effective medical therapy for hypoglycaemia.