A primary hepatic gastrinoma accompanied by hyperplasia of multi-nodular Brunner's glands

Abstract

Background: Primary hepatic gastrinoma causing severe ulcerogenic syndrome is extremely rare. Herein, we report a case of primary hepatic gastrinoma accompanied by hyperplasia of multi-nodular Brunner's glands in a patient who instead, preoperatively, was suspected of having multiple duodenal gastrinomas and hepatic metastasis.

Case presentation: A 57-year-old woman consulted a clinic complaining of melena, intermittent abdominal pain, diarrhea, and vomiting which had persisted for about 3 years. Six months before her presentation, she underwent segmental resection of the jejunum for acute peritonitis due to the spontaneous jejunal perforation. A blood test revealed that her serum immunoreactive gastrin (IRG) level was 12,037 pg/mL. Subsequently, she was transferred to our hospital. On computed tomography (CT), a hypervascular tumor of 23 mm in the segment 5 (S5) region of the liver was visualized. A selective arterial secretagogue injection test (SASI test) was performed twice. The first SASI test revealed that the hepatic tumor was a gastrinoma, and there was no gastrinoma in the duodeno-pancreatic region. Additionally, somatostatin receptor scintigraphy only visualized the tumor in the liver. However, the second SASI test, which was performed during the administration of a proton pump inhibitor and a somatostatin analog (octreotide acetate), revealed that there may have been gastrinomas existing not only in the liver but also in the upper part of the duodenum or the head of the pancreas. Duodenal endoscopy revealed multiple submucosal tumors in the first and the second portion of the duodenum, although a pathological examination of biopsied specimens obtained from the duodenal lesions was negative for malignant cells. Multiple endocrine neoplasia type 1 (MEN1) was excluded from her family history, and serum levels of both intact parathyroid hormone (iPTH) and calcium were within normal ranges. An anterior segmentectomy of the liver and pancreas-preserving total duodenectomy were performed on September 9, 2013. Postoperatively, her serum immunoreactive gastrin level decreased to less than 50 pg/mL. Pathological study of the resected specimens revealed a gastrinoma in the liver, but no gastrinoma in the duodenum. Interestingly, the duodenal submucosal tumor-like lesions were hyperplastic Brunner's glands. Postoperatively, she has been well without recurrence of hypergastrinemia for 4 years.

Conclusion: We report a case of primary hepatic gastrinoma in a patient who has been cured for 4 years postoperatively. The diagnosis was somewhat difficult due to the coexisting, multiple hyperplastic Brunner's glands of the duodenum mimicking the submucosal neuroendocrine tumors, which might have developed due to long-term hypergastrinemia.

Keywords: Hypergastrinemia; Hyperplasia of Brunner’s glands; Pancreas-preserving total duodenectomy; Primary hepatic gastrinoma; Selective arterial secretagogue injection test; Somatostatin receptor scintigraphy.

Fig. 1

Preoperative imaging findings. a Abdominal contrast-enhanced computed tomography (CT) showing a hypervascular tumor in the posteroinferior segment of the right hepatic lobe (arrow). b Magnetic resonance imaging (MRI) showing a hypointensity tumor on T2-weighted imaging (arrow). c Somatostatin receptor scintigraphy (SRS) showing abnormal uptakes in the liver mass. d Upper gastrointestinal endoscopy showing several submucosal tumor-like lesions in the first to the second portion of the duodenum

Fig. 2

The selective arterial secretagogue injection test (SASI) test. a The first SASI test showed a significant elevation of the serum IRG level from 6427 to 79,160 pg/mL at 40 s after the stimulation of the right hepatic artery. b The second SASI test showed an elevation of the serum IRG level from 56 to 130 pg/mL at 40 s after the stimulation of the gastroduodenal artery

Fig. 3

Resected specimen. a A solid mass, 13 mm in diameter, was observed in the liver. b Multiple submucosal tumor-like lesions were observed in the duodenum

Fig. 4

Histopathological examinations. a Hematoxylin and eosin staining of the liver mass showing a funicular, solid, and tubular increase of small cube-shaped, atypical cells (×200 magnification). b Immunostaining for chromogranin A of the liver mass was positive (×200 magnification). c Immunostaining for gastrin in the liver mass was positive (×200 magnification). d Hematoxylin and eosin staining of the duodenum showing hyperplasia of the Brunner’s glands (×200 magnification). e Immunostaining for chromogranin A in the duodenum was negative (×100 magnification). f Immunostaining for gastrin in the duodenum showing a small gathering of gastrin-positive cells (×200 magnification)