Primary Hepatic Gastrinoma as an Unusual Manifestation of Zollinger-Ellison Syndrome

Abstract

We report a rare case of primary hepatic gastrinoma. A 77-year-old woman exhibited continuous watery diarrhea for 8 months and weight loss. Bacterial cultures of the stools were negative and colonoscopy revealed no abnormalities. Esophagogastroduodenoscopy showed severe reflux esophagitis and multiple duodenal erosions. Computed tomography and magnetic resonance imaging detected two solid masses measuring <2 cm in diameter in the right lobe of the non-cirrhotic liver. Microscopically, the tumor was consistent with neuroendocrine tumor (grade 2) with abundant gastrin-immunoreactive cells. Endoscopic ultrasound detected no other alternative primary source of an endocrine tumor. The serum gastrin levels exceeded 40,000 pg/ml in the absence of H₂ receptor antagonist and proton pump inhibitor administrations. Based on an arterial stimulation and venous sampling test, the patient was diagnosed as primary gastrinoma of the liver. Our findings demonstrated the presence of Zollinger-Ellison syndrome in a patient who was subsequently cured by surgical resection of the liver tumors.

Case of primary hepatic gastrinoma: Diagnostic usefulness of the selective arterial calcium injection test

“…SACI tests were regarded as not being useful for the diagnosis of primary hepatic gastrinomas. Because the calcium injected into arteries flows into the liver via the portal vein and stimulates secretion of gastrin, calcium injection into any artery was thought to increase serum gastrin concentration . We found, however, that the concentration of gastrin in the hepatic vein did not increase after calcium injection into any artery except for the proper hepatic arteries.…”

“…Because the calcium injected into arteries flows into the liver via the portal vein and stimulates secretion of gastrin, calcium injection into any artery was thought to increase serum gastrin concentration. 7,10 We found, however, that the concentration of gastrin in the hepatic vein did not increase after calcium injection into any artery except for the proper hepatic arteries. These findings suggest that the amount of calcium that reached the hepatic tumor via the portal vein was too small to increase the concentration of gastrin in the hepatic vein.…”

Nowotwory neuroendokrynne żołądka i dwunastnicy z uwzględnieniem gastrinoma (zasady postępowania rekomendowane przez Polską Sieć Guzów Neuroendokrynnych)

“…-in sporadic ZES gastrinomas may occur occasionally in the liver (< 1%) and liver/biliary ducts [20,25,26,27], which has recently been demonstrated also for patients with MEN-1/ZES [28]. Zollinger-Ellison Syndrome should be suspected in patients with ulcer disease with [20]: -multiple ulcers of the upper part of the gastrointestinal tract, with unusual location, -relapses after treatment, -concomitant severe oesophagitis, -negative H. pylori test results, -complications of the disease (gastrointestinal tract perforation, bleeding), -diarrhoea, -thickening of the gastric folds (present in 92% of ZES patients).…”

Neuroendocrine Tumors of the Hepatobiliary Tract

“…Primary intrahepatic gastrinoma is a very rare neoplasm of the liver. Relatively few reports have convincingly documented gastrinoma primary to the liver (Smith and Auldist 1984;Larriva-Sahd et al 1992;Inoue et al 1993;Moriura et al 1993;Smyrniotis et al 1994;Krishnamurty et al 1996;Tiomny et al 1997;Kehagias et al 1999;Bello Argues et al 2001;Chien et al 2001;Deol et al 2003;Shibata et al 2006;Ishikawa et al 2008;Denève et al 2009;Rascarachi et al 2009;Tsalis et al 2011;Naoe et al 2012;Ogawa et al 2014). It is of special interest because it may, as gastrinomas in other locations, be associated with Zollinger-Ellison syndrome (Naoe et al 2012).…”