A Rare Case of Zollinger-Ellison Syndrome and Pancreatic Adenocarcinoma in a Patient with Multiple Endocrine Neoplasia Type 1

Abstract

A 50 year-old male presented to the hospital with a 2-week history of abdominal pain. He also referred chronic diarrhea described as alternating between watery and pasty. Medical history was remarkable for MEN1 diagnosed at 14 years of age after he was found with hyperplasia of the parathyroid glands with elevated calcium levels for which he underwent a partial parathyroidectomy. History is also significant for a pituitary adenoma treated with bromocriptine for 1 year. Family history is pertinent for MEN1 in his mother and maternal grandfather. During hospitalization, a CT scan showed a cystic lesion at the pancreatic tail. An MRI showed a lesion at the head of the pancreas and a cystic, non-enhancing pancreatic tail lesion. Upper endoscopy was remarkable for multiple ulcerated lesions in the duodenum (fig 1). Gastrin levels were elevated. A somatostatin receptor imaging study disclosed no uptake. The patient was referred to an interventional gastroenterologist after discharge. By this time, he was presenting with jaundice and pruritus and chemistry was remarkable for a cholestatic pattern. An endoscopic ultrasound disclosed a pancreatic tail cyst, a pancreatic tail mass (fig 2), and a pancreatic head mass causing obstruction and dilation of the common bile duct (CBD). Pathologic diagnosis for the pancreatic tail cyst and mass showed hyperchromatic cells with chromogranin and synaptophysin (fig 3) and adenocarcinoma of the pancreatic head mass. An ERCP was done with stent placement at the CBD. The patient underwent a Whipple procedure with distal pancreatectomy and duodenectomy and was started on adjuvant chemotherapy with gemcitabine for an expected total duration of 6 months. MEN1 is characterized by pituitary tumors, parathyroid adenomas, and pancreatic neuroendocrine tumors (pNETs). This is the rare case of a patient with both a pNET causing Zollinger-Ellison syndrome (ZES) and a pancreatic adenocarcinoma in the setting of MEN1. A literature review disclosed one similar case in a 46 year-old patient with MEN1, ZES and a pancreatic adenocarcinoma. In our patient, pathology disclosed markers characteristic of a pNET. However, somatostatin receptor imaging did not show uptake in the pancreas. It is important to always consider the presence of a neuroendocrine tumor in patients with MEN1 in spite of negative imaging since earlier intervention with resection will have a greater impact in the patient's clinical outcome and reduce morbidity and mortality.1446_A.tif Figure 1: Erosive mucosa and multiple ulcers in the second and third portion of the duodenum.1446_B.tif Figure 2: Pancreatic tail mass distal to pancreatic cyst.1446_C.tif Figure 3: Pancreatic tail mass pathology positive for chromogranin and synaptophysin.