A diagnostic dilemma of hormone-secreting paraganglioma presenting as secondary diabetes

Abstract

Paragangliomas are rare neuroendocrine neoplasms that arise from chromaffin cells. They most commonly arise in adrenal medulla and are called as pheochromocytoma. About 10% of paragangliomas arise at extra-adrenal sites. Of the functioning extra-adrenal paragangliomas, majority present with intermittent hypertensive attacks. Very rarely, paragangliomas secreting other hormones including adrenocorticotropic hormone, endorphins, pancreatic polypeptide, androgens, and insulin have been reported. Patients should be evaluated with imaging studies along with appropriate hormonal assays keeping in mind the variety of hormones paragangliomas may secrete in turn leading to diagnostic dilemmas. This case report describes a patient with uncontrolled high blood glucose levels and an abdominal mass initially suspected for a glucagonoma which was later discovered to be a functioning retroperitoneal paraganglioma.