Endocrine Abstracts

Introduction: Hemangiopericytomas (HPC) are rare vascular tumours that may be associated with hypoglycaemia. Here we report a case of HPC with multiple metastases, accompanying severe recurrent hypoglycaemia due to the combination of different aetiologies.

Case report: A 21 year-old woman was hospitalised due to loss of consciousness. The patient has been diagnosed as HPC by tibia mass biopsy 1 year ago. Tumour embolization was made several times. On admission, serum glucose was 42 mg/dl and serum insulin and C-peptide levels were suppressed. GH and cortisol response to hypoglycaemia were found insufficient. Basal ACTH was below 5 pg/ml. IGF2 was 25 ng/ml (normal range 116–358 ng/ml) and IGF2 was 823 ng/ml (normal range 288–736 ng/ml). The IGF2/IGF1 ratio was 32.9 (normal range <10), which indicates unregulated production of IGF2. Thorax and abdomen CT imaging demonstrated multiple metastatic lesions in the liver, lung, abdominal cavity, pelvis and bone. MRI revealed a microadenoma in the pituitary gland. In this case, we suggest that hypoglycaemia was associated with the combination of adrenal insufficiency secondary to deficient ACTH secretion, abnormal production of IGF2 and diminished hepatic glucose production due to the liver metastases. Because of recurrent severe hypoglycaemic attacks, a continuous glucose infusion was required to maintain normoglycaemia. Prednisolone 40 mg/day was started and a dramatically quick response was observed. Hypoglycaemic attacks were resolved immediately and 20 mg/day prednisolone was needed as maintenance therapy to prevent recurrent hypoglycaemia.

Conclusions: HPC associated hypoglycaemia is a paraneoplastic syndrome usually related with excessive production of IGF2 or partially processed forms of pro-IGF2. Hypoglycaemia can also develop by combinations of different factors such as adrenal insufficiency that we observed in the present case. The possibility of other factor must also take into consideration evaluating and treating the hypoglycaemic attacks in patients with mesenchymal tumours.