Endocrine Abstracts

We previously reported a case of non islet cell tumour hypoglycaemia (NICTH) due to increased ‘big’-IGF2 production in a lady with haemangiopericytoma (HAP). We now describe the challenges in managing this lady who had recurrent, disabling hypoglycaemia requiring frequent hospitalisation.

This patient presented with symptomatic hypoglycaemia 15 years after the diagnosis of parasaggital HAP. At presentation she had tumour recurrence with liver and brain metastases.

Surgical resection was not an option due to extent of disease, but she received chemotherapy to reduce tumour bulk. When this failed to alleviate symptoms, we commenced prednisolone 10 mg OD to which she responded well for a few months. Her hypoglycaemia returned, prompting addition of recombinant GH (rGH) to her therapy. We limited the use of glucagon for treatment of hypoglycaemic emergencies.

Despite combination therapy hypoglycaemia relapsed, and hepatic artery embolization was undertaken to decrease tumour bulk. Diazoxide was considered, but was abandoned due to poor tolerance. She had s.c. somatotropin briefly, which was stopped when octreotide scanning showed no tracer uptake.

As her condition deteriorated, debulking surgery or localised radiotherapy for liver metastases became impossible. Hypoglycaemia in the last days of her life was managed with i.v. dextrose.

Hypoglycaemia due to NICHT is difficult to treat. Complete resection of the tumour cures hypoglycaemia, but when impossible, reduction of tumour bulk will help. Glucocorticoids and rGH form the mainstay of medical therapy. Glucocorticoids provide symptom relief by stimulating gluconeogenesis. How rGH works is not understood, but it can be used alone or in combination with steroids.

This case is interesting as much for the challenges of management as for the rarity of the diagnosis.