Multiple Endocrine Neoplasia Type 1 Syndrome: A Case Report and Review of Literature

Abstract

Multiple endocrine neoplasia type 1 (MEN1) or Wermer's syndrome is a genetic disease characterized by involvement of multiple endocrine glands, primarily involving parathyroid, pancreas, and pituitary. Other additional features include foregut carcinoids; non-functioning adrenal tumors; and skin lesions such as lipomas, collagenomas, and angiofibromas. Here, we describe our experience in managing a patient who presented to us with spontaneous episodes of hypoglycemia and was diagnosed with insulinoma. Detailed clinical and biochemical evaluation unraveled the diagnosis of MEN1 in the patient and her family members who constituted a large kindred. This case highlights the importance for evaluation of MEN1 in a patient or his/her family members in the setting of clinical and biochemical suspicion. In addition, we have also discussed the utility of the latest diagnostic and therapeutic modalities for management of MEN1.