Endocrine Abstracts

Background: Non-islet-cell tumour hypoglycaemia (NICTH) is a rare paraneoplastic syndrome caused by an extra pancreatic tumour(1). Solitary fibrous tumour (SFT), amongst other tumours, is one of the rare causes of NICTH. We report a case of NICTH who presented with recurrent hypoglycaemias and was identified to have a fibrous tumour as part of the work up for his symptomatic hypoglycaemias.

Case Summary: A 75-year male had presented to the emergency department with severe symptomatic hypoglycaemia with a blood glucose of 0.9. His past medical history included hypertension and Gout. He was not on any medication that could have caused or contributed to a hypoglycaemia. His initial biochemical workup showed subnormal insulin and c peptide levels with low blood glucose reading (physiological response) in the presence of negative ketones and negative sulfonylurea screen. He also had a CT scan done as part of the workup for recurrent hypoglycaemia that showed a well-defined heterogeneously enhancing mass in the pelvis with no evidence of local or distant metastases. He was referred for surgery of his pelvis mass, the histology of which showed a SFT (immunohistochemistry positive for CD34 and STAT6, negative for S100, Sox10, CD117, DOG1, SMA and Desmin). Reassuringly, his symptoms of hypoglycaemias resolved completely post-surgery.

Conclusion: We present a case of NICTH secondary to SFT. The tumour secretes high molecular weight IGF-II(2) which has structural and biochemical homology with insulin resulting in hypoglycaemias(3). The management of NICTH is complete resection of the extra pancreatic tumour which eventually leads to resolution of the symptoms as happened in our case(4).

References: 1. Pelosof LC, Gerber DE. Paraneoplastic Syndromes: An Approach to Diagnosis and Treatment. Mayo Clin Proc. 2010 Sep;85(9):838–54. 2. Phillips LS, Robertson DG. Insulin-like growth factors and non-islet cell tumor hypoglycemia. Metabolism. 1993 Sep;42(9):1093–101.3. Khowaja A, Johnson-Rabbett B, Bantle J, Moheet A. Hypoglycemia mediated by paraneoplastic production of Insulin like growth factor–2 from a malignant renal solitary fibrous tumor – clinical case and literature review. BMC Endocr Disord. 2014 Dec 17;14(1):49.4. Bodnar TW, Acevedo MJ, Pietropaolo M. Management of Non-Islet-Cell Tumor Hypoglycemia: A Clinical Review. J Clin Endocrinol Metab. 2014 Mar 1;99(3):713–22.