Insulinoma in a patient with Congenital adrenal hyperplasia

Abstract

Insulinomas are functional pancreatic neuroendocrine tumors that can cause hypoglycemia. Congenital adrenal hyperplasia (CAH) is a disease that can have mutations in a variety of mutations that comprise cortisol synthesis and require certain patients to require cortisol supplementation to prevent hypoglycemia. We report a case presentation of a patient with CAH and migraines who develops an insulinoma with neuroglycopenic symptoms. His hypoglycemia was treated with stress dose steroids and fluid resuscitation before undergoing a 72-h fast to confirm the diagnosis of an insulinoma. The patient then underwent a robotic distal pancreatectomy with postoperative stress dose steroids and had no further hypoglycemic complications on his home regimen of hydrocortisone supplementation. As his neuroglycopenic symptoms were initially thought to be migraines exacerbating his CAH, this case illustrates the need to keep a broad differential for hypoglycemia in patients with CAH. Insulinomas should be in that differential and the appropriate biochemical testing and imaging should be performed.