Surgical Management of Zollinger-Ellison Syndrome; State of the Art

A Perplexing Case of Abdominal Pain That Led to the Diagnosis of Zollinger-Ellison Syndrome

“…CT and MRI are both highly accurate in detecting lesions greater than 3 cm; however, accuracy decreases for tumors that are smaller than 2 cm in size (studies have reported around 42–56% sensitivity of CT scans and 25–83% sensitivity for MRI) [18, 19]. Gastrinomas express receptors for somatostatin, allowing SRS scans to be utilized for tumor detection with a higher sensitivity than CT/MRI (reported to be around 80–85%) [13, 19]. Recently, EUS has also become an important imaging study and allows for the identification of pancreatic tumors as small as 2–5 mm in diameter, with sensitivity reaching up to 87% [19–21].…”

“…However, pancreatic tumors are typically more aggressive and are more likely to metastasize to the lymph nodes, liver, and/or bone than are duodenal tumors [10, 11]. Around 80% of gastrinomas are detected within the gastrinoma triangle, defined as the interface between the confluence of the cystic duct and common bile duct, the junction of the second and third portions of the duodenum, and the junction of the neck and body of the pancreas [12, 13]. Patients with ZES typically present with nonspecific GI symptoms, such as abdominal pain, nausea, vomiting, and chronic diarrhea [2].…”

Metastatic gastrinoma in the breast mimicking primary solid papillary carcinoma

“…These tumors may result in Zollinger-Ellison Syndrome which is characterized by hypergastrinemia, gastric acid hypersecretion, peptic ulcer disease, abdominal pain, nausea, heartburn and often diarrhea [4]. Gastrinomas may occur sporadically or in the setting of multiple endocrine neoplasia 1/MEN1 (roughly 20% of individuals with gastrinomas have MEN1) [4,5]. Between 60-90% of gastrinomas are clinically malignant with metastases to the lymph nodes, liver, or other distant sites at diagnosis [5,6,7].…”

“…It usually metastasizes to the liver or regional lymph nodes [3,4]. We herein report a case of metastatic gastrinoma to the breast.…”

Italian Association of Clinical Endocrinologists (AME) position statement: a stepwise clinical approach to the diagnosis of gastroenteropancreatic neuroendocrine neoplasms

“…Accurate localization of the tumor can result in complete surgical resection, decreased rate of developing lymph node metastases, and increasing survival [88, 222, 224–226] (Fig. 10).…”