Man with sporadic MEN1 and Zollinger Ellison syndrome: A case Report

Background: MEN1 is a rare hereditary syndrome which manifests a variety of endocrine and non-endocrine neoplasms and lesions. Introduction: A 59 year-old male with good clinical status was referred to our hospital due to chronic episodes of diarrhea. Imaging studies revealed multiple polyps in various parts of the colon, a pancreatic tail tumor, 2 hepatic lesions, bilateral adrenal hyperplasia and pituitary microadenoma.The parathyroid sestamibi scan showed abnormal concentration of radioactivity. He was submitted to peripheral pancreatectomy/ splenectomy. Biopsy revealed a well differentiated NF, G2 pNET. Blood examination showed high PTH and gastrin levels. Gastroscopy suggested Zollinger Ellison syndrome. Genetic examination demonstrated a pathogenic MEN1 mutation and further exams supported this diagnosis. His brother was also tested and found negative for any gene mutation. He was treated postoperatively with Everolimus and RFA for local hepatic control. On progression he was treated with Somatulin Autogelim monthly. His hypergastrinemia subsided and he remains asymptomatic on close follow up.