SUN-LB067 Uncontrolled Diabetes with Severe Ketoacidosis: Blame It on the Pheochromocytoma!

Abstract

Background: Pheochromocytomas are rare catecholamine secreting tumors that arise from chromaffin cells of the adrenal medulla and sympathetic ganglia. Typical symptoms include paroxysmal hypertension, headache, and palpitations. Hyperglycemia is seen in 35-50% of these patients. There are a few case reports depicting the rare presentation of diabetic ketoacidosis (DKA) precipitated by a catecholamine-secreting tumor. We present a patient with recurrent DKA in the setting of a pheochromocytoma. Clinical Case: A 57-year-old woman with a history of breast cancer, hypertension, adrenal mass, and uncontrolled diabetes mellitus presented with severe DKA after taking oral steroids. Her review of systems noted intermittent pounding headaches, blurred vision, dizziness, unintentional weight loss, palpitations, and fluctuating blood pressure (BP). She had an episode of DKA 6 months prior. She was treated in the ICU with intravenous fluid and insulin with resolution of DKA. Subsequently, she acutely became severely hypertensive. BP was uncontrolled despite aggressive intravenous anti-hypertensive medication. Pertinent labs included hemoglobin A1c (HbA1c) 9.1%, GAD-65 Ab <5 IU/mL, fasting C-peptide 0.81 ng/mL, plasma free total metanephrines 29073 pg/mL(<205) and 24 hour urine total metanephrines 34633 mcg (149-603). A CT scan of the abdomen showed a 7.8 cm right adrenal mass with heterogeneous internal enhancement. Her BP was stabilized with clonidine and doxazosin and she was discharged on these medications and insulin. After appropriate alpha and beta blockade, she underwent right adrenalectomy at another hospital. Postoperatively her BP has been controlled off alpha-blockade and her diabetes has been controlled on metformin alone. Conclusion: This case illustrates the causative role of a pheochromocytoma in a patient with hyperglycemia and severe ketoacidosis. Epinephrine secreted by a pheochromocytoma inhibits insulin secretion by stimulation of alpha-adrenergic receptors and induces glucagon secretion by stimulation of beta and alpha-adrenergic receptors in the pancreas. In addition, stimulation of these receptors promotes glycogenolysis and gluconeogenesis in the liver and activates lipolysis contributing to insulin resistance. In a predisposed patient, these factors may lead to DKA. In a patient who presents with DKA and paroxysmal or uncontrolled hypertension, the diagnosis of pheochromocytoma should be considered. Unless otherwise noted, all abstracts presented at ENDO are embargoed until the date and time of presentation. For oral presentations, the abstracts are embargoed until the session begins. s presented at a news conference are embargoed until the date and time of the news conference. The Endocrine Society reserves the right to lift the embargo on specific abstracts that are selected for promotion prior to or during ENDO.