CASE 16880 Published on 13.07.2020

Multicentric gastrinoma with secondary Zollinger-Ellison Syndrome.

Section

Abdominal imaging

Case Type

Clinical Cases

Authors

Vicaría Fernández, Iván; Unzué García-Falcés, Guillermo; Alberdi Aldasoro, Nerea; López Sala, Paul; De Llano Ibisate, Loreto Ana; Laxe Vidal, Tamara; Lajusticia Andrés, Héctor.

Radiology Department. Complejo Hospitalario de Navarra; C/Irunlarrea 3; 31008, Pamplona; Navarra; Spain

Patient

79 years, male

Categories
Area of Interest Abdomen, Gastrointestinal tract ; Imaging Technique Abdomen, Gastrointestinal tract
Clinical History

A 79-year-old male with epigastric abdominal pain and rectal bleeding, followed by haematochezia and hypotension. He was submitted to an angio-CT scan that depicted active arterial bleeding at duodenum-proximal jejunum. The therapeutical approach made was the combination of arterial embolization and omeprazole + somatostatin endovenous perfusion. Gastrin levels reached 1786pg/ml.

Imaging Findings

Abdominal angio-CT depicted two small intramural high attenuation nodules of 13 and 7mm, located on the horizontal duodenal portion and on the pyloric sphincter associating important gastric wall thickening.

In addition, CT revealed an ongoing active arterial bleeding at proximal jejunum. Blood supply was received from a jejunal branch of the superior mesenteric artery. Angio-CT scan showed the active bleeding as vivid intraluminal enhancement on the arterial phase because of contrast extravasation that was not present on the non-contrast images. Finally, in the venous phase we found an increasing quantity on contrast extravasation, which was a useful finding to confirm the presence of an active arterial bleeding ongoing.

An Octreoscan was also performed and depicted the enhancement of the lesions due to the presence of somatostatin receptors on them. They are shown in black on the conventional gammagraphy images and yellow on the SEPCT-CT images below.

Discussion

Gastrinoma is the most common gastrointestinal (GI) neuroendocrine tumour (NET, 62-65%) [1]. Gastrin is produced by G cells, which are mostly located in the antral gastric region but they can also be occasionally found in the pancreas and duodenum.

When a gastrinoma produces high blood gastrin levels, typically beyond 1000pg/ml, it develops different clinical manifestations due to those high hormone levels. Zollinger-Ellison Syndrome (ZES) is a clinical syndrome secondary to the effects triggered by hypergastrinemia. These effects come from gastric parietal cells hyperstimulation, which runs into an excessive clorhidric acid production that develops the clinical manifestations referred below in B.

ZES is most commonly produced by multicentric gastrinomas, and these, in turn, are most common in type 1 multiple endocrine neoplasia (MEN-1) context [2].

The starting clinical unspecificity brings as to an issue which comes to a 5 to 7 years diagnostic delay since the symptomatology beginning. However, the typical clinical presentation consists on different combinations of the following symptoms: abdominal pain, nausea, vomiting, dyspepsia, gastroesophageal reflux, ulcers, GI bleeding, weight loss or chronic diarrhoea [3].

Imaging is essential in order to find the hypervascular gastrin producer nodules whose height is usually <1cm [3]. In consequence, in many cases these lesions neither can be endoscopically seen nor a biopsy can be performed.

We must convey the requesting physician the location, size, amount and image characteristics of the nodules and other possible findings associated such as gastric wall thickening, telangiectasis or GI bleeding, also pointing the accurate bleeding point.

 

GI NETs are depicted on contrast-enhanced CT scan as high attenuation, intramural and spherical lesions located mostly on the duodenum. The ideal scenario would be having pathological diagnosis but this is uncommon so that first-choice imaging technique to perform is an angio-CT scan. Therefore, in many cases diagnosis is made by combining clinical and imaging features.

 

ZES first-line treatment is surgery [1, 4], but in non-operable individuals, another option is proton-pump inhibitors. However, the ulcer-peptic disease produced is commonly refractory to medical treatment. Other options could be endoscopic or arterial embolization in case of GI bleeding. Imaging gives important information to choose the most suitable treatment.

 

Gastrinoma is the most common GI NET and ZES is a clinical syndrome due to high gastrin levels. Angio-CT scan is the first-choice image technique to perform and depending on its findings we can make an appropriate diagnosis and select the most suitable treatment.

Differential Diagnosis List
Multicentric gastrinoma with secondary ZES.
Neuroendocrine tumours
Metastases: from primary hypervascular tumours; melanoma, breast, hypernephroma, sarcomas.
Heterotopical tissues: heterotopic gastric mucosae, intrapancreatic accessory spleen
Telangiectasis.
Final Diagnosis
Multicentric gastrinoma with secondary ZES.
Case information
URL: https://www.eurorad.org/case/16880
ISSN: 1563-4086
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