Primary hepatic gastrinoma being diagnosed preoperatively: a case report and literature review

Abstract

Background A majority of gastrinomas causing Zollinger–Ellison syndrome are located in the duodenum or pancreas. Primary hepatic gastrinomas are rare and difficult to diagnose. We report a rare case of primary hepatic gastrinoma, which could be diagnosed preoperatively. Case presentation A 29-year-old man with a 55-mm tumor in segments 5 and 6 (S 5/6) of the liver was admitted to our hospital. After thorough investigations, he was treated for a suspected inflammatory pseudotumor and advised to undergo routine follow-up. Two years later, he revisited our hospital with a complaint of abdominal pain, vomiting, and diarrhea. Upper gastrointestinal endoscopy revealed multiple duodenal ulcers. His serum gastrin level was 2350 pg/mL (normal: 37–172 pg/mL), suggesting Zollinger–Ellison syndrome. Abdominal computed tomography showed a 78-mm hypervascular tumor with cystic degeneration in the S 5/6 region of the liver, with a potential to increase over time. The tumor showed hypointensity on T2-weighted and hyperintensity on diffusion-weighted abdominal contrast-enhanced magnetic resonance imaging. Somatostatin receptor scintigraphy (SRS) only detected a hepatic tumor. No tumors in the gastrinoma triangle were detected by endoscopic ultrasonography. Hence, selective arterial calcium injection (SACI) test was performed to determine the location of the gastrinoma. The serum gastrin concentration increased from 4620 pg/mL to 23,600 pg/mL at 20 s after calcium gluconate injection into the proper hepatic artery. Conversely, no effect on serum gastrin level was observed after the injection into any other arteries. Extended right hepatic lobectomy and cholecystectomy were performed after percutaneous transhepatic portal vein embolization. A histopathological examination of the liver tumor revealed a gastrinoma. The patient’s serum gastrin concentration on postoperative day 1 decreased to 65 pg/mL. Conclusion We report a surgical case of primary hepatic gastrinoma correctly diagnosed preoperatively. The patient underwent extended right hepatic lobectomy, resulting in a histological definitive diagnosis of primary hepatic gastrinoma.

A Rare Case of Hepatic Gastrinoma

“…The second and third portions of the duodenum, the pancreatic head, and the hepatoduodenal ligament act as its anatomic boundaries. Hepatic gastrin-secreting tumors are thus a rare finding, with less than 40 cases reported previously in the literature [ 1 ]. Regardless of location, all gastrinomas can manifest as ZES, presenting as either peptic ulcer disease, diarrhea, or gastroesophageal reflux disease that is often refractory to conventional management.…”

“…Zollinger-Ellison syndrome (ZES) is caused by hypersecretion of gastrin by neuroendocrine tumors, known as gastrinomas, found most commonly in the stomach, duodenum, or pancreas [ 1 ]. Although ZES is commonly discussed as a cause of upper gastrointestinal (GI) bleeding in the literature, it is rarely seen in practice.…”

Primary Hepatic Neuroendocrine Tumor: A Case Report and Literature Review

“…ZES is a common presentation of gastrinomas which are gastrin-producing neuroendocrine tumors. Primary hepatic gastrinoma has been described in only 35 cases as reported in a recent literature review [ 12 ]. Additionally, she was found to have a miscarriage of her seventh week pregnancy during her ED presentation.…”

Neoadjuvant Peptide Receptor Radionuclide Therapy in a Rare Case of Pediatric Primary Hepatic Gastrinoma

“…However, primary hepatic gastrinoma is exceedingly rare (<2%) and more so in the pediatric population. [1][2][3][4] In the younger age groups, it is usually associated with type 1 multiple endocrine neoplasia (MEN1) syndrome. 177 Lu-DOTATATE is Food and Drug Administration approved for metastatic well-differentiated gastroenteropancreatic NET and has shown improvement in PFS and quality of life compared with conventional somatostatin analogues.…”

“…Gastrinoma usually occurs in the gastrinoma triangle and frequently produces liver metastases. However, primary hepatic gastrinoma is exceedingly rare (<2%) and more so in the pediatric population 1–4 . In the younger age groups, it is usually associated with type 1 multiple endocrine neoplasia ( MEN1 ) syndrome.…”