Primary hepatic gastrinoma presenting as vague gastrointestinal symptoms

Abstract

A 51-year-old Caucasian female with a 7-year history of intermittent abdominal pain and diarrhoea presented to our service. Before presentation, she had been successfully treated for Helicobacter pylori infection, but later developed new oesophageal ulcerations with exudative lesions that were positive for herpes simplex virus, and candida oesophagitis had developed. Biopsies showed chronic inactive gastritis with gastric intestinal metaplasia. MRI revealed a solid 3.4×3 cm lesion in the caudate lobe of the liver, with a 7-mm pancreatic cyst. The aspirated pancreatic cyst cytology was benign. On exploratory laporatomy, the lesion appeared confined to the caudate lobe, and a resection was performed. The pathology was consistent with a well-differentiated neuroendocrine carcinoma with vascular invasion and involvement of the liver capsule, although resection margins were negative. The patient had complete symptomatic improvement. This case re-affirms the high index of suspicion needed to make the diagnosis of gastrinoma. If caught in time, surgical removal of primary hepatic gastrinoma can be curative.

ENETS Consensus Guidelines Update for the Management of Patients with Functional Pancreatic Neuroendocrine Tumors and Non-Functional Pancreatic Neuroendocrine Tumors

“…In sporadic ZES gastrinomas are known to occasionally occur in the liver (<1%) and liver/hepato-biliary system [65;105–107], and recently also in patients with MEN1/ZES [108]. Gastrinomas in these locations, especially the duodenum can be small (<0.5 cm) and multiple, therefore sensitive imaging, especially preoperatively is mandatory [65;109;110].…”

Gastroduodenal neuroendocrine neoplasms including gastrinoma — update of the diagnostic and therapeutic guidelines (recommended by the Polish Network of Neuroendocrine Tumours) [Nowotwory neuroendokrynne żołądka i dwunastnicy z uwzględnieniem gastrinoma — uaktualnione zasady postępowania (rekomendowane przez Polską Sieć Guzów Neuroendokrynnych)]

“…Gastrinoma in the course of MEN-1/ZES: -MEN-1 is diagnosed in 20-30% of patients with ZES; In sporadic ZES, gastrin tumours occasionally occur in the liver (< 1%) and the liver/bile ducts [28,[33][34][35], which has also been recently demonstrated in patients with MEN-1/ZES [36].…”

“…-Gastrinoma w przebiegu MEN-1/ZES: -u 20-30% pacjentów z ZES rozpoznaje się MEN-1; -MEN-1/ZES w 70-100% ma lokalizację dwunastniczą, prawie zawsze guzy są mnogie; -15% ma agresywny przebieg kliniczny; -średni wiek w momencie rozpoznania: 32-35 lat (dla postaci sporadycznej: 48-55 lat); -u 45% chorych objawy ZES wyprzedzają o kilka lat hiperkalcemię objawową; -u 25% chorych z MEN-1/ZES wywiad rodzinny w kierunku MEN-1 jest ujemny. W sporadycznym ZES guzy gastrynowe mogą okazjonalnie występować w wątrobie (< 1%) oraz wątrobie/drogach żółciowych [28,[33][34][35], co niedawno wykazano także u pacjentów z MEN-1/ZES [36].…”

Zollinger–Ellison syndrome

“…This change is due to the fact that duodenal gastrinomas are frequently small(<1-cm), not seen on imaging and thus were easily missed in the early studies, and are still missed at surgery, if a routine duodenotomy isn’t performed[14,38,39,42,43]. Primary gastrinomas are uncommonly located in other intra-abdominal locations including:lymph nodes(controversial), stomach, mesentery, renal capsule, splenic hilum, omentum, ovary and in the liver/biliary tract[41,44,45,46●,47–49]. Rarely (<0.3%) primary gastrinomas may occur in extra-abdominal locations such as the heart and lung[41].…”