Multifocal Insulinoma as the Unique Presenting Feature of Multiple Endocrine Neoplasia Type 1 in an Adolescent

Have you ever heard of a de novo MEN1 mutation? I hadn't until I read this fascinating case record. Hi, I'm Sophia Schermerhorn from Cincinnati Children's, and I'm here to share this case report. This case describes a teenager who presented with recurrent hypoglycemia and what was initially thought to be a straightforward insulanoma. Initial imaging studies failed to identify a lesion, so that prompted a dodo taste scan, which showed not just one but multiple pancreatic tumors. And during surgery, 4 distinct lesions were identified and resected. Now here's where it gets interesting. Each tumor expressed a different hormone on pathology, with only one of them behaving like a true insulinoma. Genetic testing later confirmed a de novo MEN1 mutation, even though he had no family history and he had no other MEN1 associated endocrine problems. The key takeaway is that in pediatric patients, multifocal pancreatic neuroendocrine tumors should raise concern for a possible MEN1. Early recognition can both guide treatment and long-term surveillance.