Zollinger-Ellison syndrome: case report and topic review

Zollinger-Ellison syndrome (ZES) is characterized by gastrin-secreting neuroendocrine tumors (gastrinomas) in the duodenum or pancreas. It is a rare condition, most patients are diagnosed between the ages of 20 and 50, and a higher incidence in men. Gastrinomas are associated with a high risk of malignancy and the diagnosis is confirmed by the secretin stimulation test and imaging studies such as octreotide scintigraphy. We present the case of a 24-year-old man who presented with melena, asthenia, adynamia and abdominal pain, in addition to a history of peptic ulcer. Laboratory tests revealed low levels of hemoglobin and elevated levels of gastrin. Endoscopy showed a giant ulcer and subsequent surgery revealed stomach-jejunum adhesions, gastric lesions and Meckel's diverticulum. Imaging studies confirmed neuroendocrine tumor activity in the pancreas. ZES leads to sustained hypergastrinemia, causing peptic ulcers and other digestive tract complications. Gastrinomas can arise from a variety of locations and can cause peptic ulcers, malabsorption, and diarrhea. Diagnosis requires elevated fasting serum gastrin levels and hypersecretion of gastric acid. Treatment involves discontinuation of proton pump inhibitors (PPIs) before diagnostic testing and surgical resection of tumors in suitable candidates. The diagnosis of ZES can be complicated due to the unreliability of the assays and the need for secretin testing. Surgical resection is recommended for sporadic gastrinomas without metastasis, while medical treatment may be necessary for postsurgical residual hyperacidity. Patients should undergo imaging studies for tumor localization and regular monitoring for complications and recurrences.

Análogos de somatostatina radiomarcados en el acercamiento diagnóstico del síndrome de Sjögren: Imágenes moleculares aportando a los criterios clínicos y paraclínicos hoy conocidos / Radiolabelled somatostatin analogues in the diagnostic approach to Sjögren's syndrome: molecular imaging contributing to the current clinical and para-clinical criteria

RESUMEN El diagnóstico del síndrome de Sjögren se basa en los criterios del consenso americano y europeo (AECG), sin embargo, en muchas oportunidades no se alcanza a detectar el compromiso glandular o extraglandular. Presentamos la evidencia de la utilidad de la gammagrafía con los análogos de somatostatina radiomarcados como prueba novedosa en el acercamiento diagnóstico al compromiso glandular y extraglandular del síndrome de Sjögren.

ABSTRACT Sjögren syndrome is diagnosed using American European Consensus Group (AECG) criteria, although frequently these criteria are not enough to detect the glandular and extra-glandular compromise. Evidence is presented on the use of whole body somatostatin scintigraphy as a novel probe in the diagnostic approach to the glandular and extra-glandular compromise in Sjögren s syndrome.

Diagnostic work-up of gastroenteropancreatic neuroendocrine tumors

Neuroendocrine tumors are a heterogeneous group of malignancies that present a diagnostic challenge. The majority of patients (more than 60%) present with metastatic disease at diagnosis. The diagnosis is based on histopathology, imaging, and circulating biomarkers. The histopathology should contain specific neuroendocrine markers such as chromogranin A, synaptophysin, and neuron-specific enolase and also an estimate of the proliferation by Ki-67 (MIB1). Standard imaging procedures consist of computed tomography or magnetic resonance imaging together with somatostatin receptor scintigraphy. 68Ga-DOTA-octreotate scans will in the future replace somatostatin receptor scintigraphy because they have higher specificity and sensitivity. Other positron imaging tomographic scanning tracers that will come into clinical use are 18F-DOPA and 11C-5HTP. Neuroendocrine tumors secrete many different peptides and amines that can be used as circulating biomarkers. The most useful general marker is chromogranin A, which is both a diagnostic and prognostic marker in most neuroendocrine tumors. However, there is still a need for improved biomarkers for early detection and follow-up of patients during treatment. In addition, molecular imaging can be further developed for both detection and evaluation of treatment.

Gammagrafía con receptores de somatostatina en un caso de carcinoma medular de tiroides / Somatostatin receptor scintigraphy for thyroid medullary carcinoma: a case report

La gammagrafía con radiotrazadores que tienen afinidad por los receptores de somatostatina se ha convertido en metodología eficaz para el diagnóstico y estadificación de los tumores neuroendocrinos. Se presenta un caso en el cual el procedimiento radioisotópico muestra su efectividad en la localización del tumor primario.

Somatostatin receptor scintigraphy has become an important tool for diagnosis and evaluation of neuroendocrine tumors. This case report shows about the importance of the radionuclide procedure for the localization of the primary tumor.

A Case of Zollinger-Ellison Syndrome with Gastrinoma Localized by 111In-Pentetreotide Scan / 대한핵의학회잡지

In patient with Zollinger-Ellison syndrome, it is difficult to localize gastrinoma because the tumor is frequently small and multiple. However, accurate localization of the tumor is important for the treatment. Among various imaging modalities, somatostatin receptor scintigraphy (SRS) has been recognized to be the most sensitive tool for the detection of neuroendocrine tumors such as gastrinomas based on the presence of high-affinity binding sites for somatostatin. Recently, we experienced a case of Zollinger-Ellison syndrome caused by gastrinomas which was localized by SRS. This is the first case report of gastrinoma detected by SRS in Korea. SRS can facilitate tumor detection in patient with Zollinger-Ellison syndrome and should be considered as the first-line diagnostic method in the early course of the disease.