Multiple endocrine neoplasia type 1 (MEN1) is a rare autosomal dominant disease caused by a mutation in the MEN1 gene. We present a 65-year-old man with MEN1 who has primary hyperparathyroidism, microprolactinoma, meningioma and gastrinoma. He had undergone parathyroidectomy followed by tumour excision of meningioma. The duodenal gastrinoma lesion was inoperable as it was close to the superior mesenteric artery with high surgery risk. Medical therapy with octreotide LAR had been initiated and showed good biochemical response as well as disease progression control. Chemoembolization was proposed if the duodenum lesion reduces in size on maintenance treatment with octreotide LAR. This case highlights the challenges in managing this rare condition and octreotide LAR has shown to be effective in controlling the disease progression in MEN1 with inoperable gastrinoma
meningioma ; octreotide ; gastrinoma

Of the various endocrine tumors affecting the pancreas, insulinomas and gastrinomas are the most common. In order to facilitate surgery, the localization of tumor is important. However, at the first time of presentation, the localization of tumor was often difficult by CT or ultrasonography because the tumor was relatively too small. The introduction of endoscopic ultrasonography has allowed high-resolution imaging of the pancreas that distinguishes structures as small as 2 to 3 mm in diameter. Thus, it has became easier to detect the lesion site of pancreatic endocrine tumor by endoscopic ultrasonography. We report the 5 patients who had pancreatic insulinoma which were detected and localized by endoscopic ultrasonography.
Endosonography* ; Gastrinoma ; Humans ; Insulinoma* ; Pancreas ; Ultrasonography

Of the various endocrine tumors affecting the pancreas, insulinomas and gastrinomas are the most common. In order to facilitate surgery, the localization of tumor is important. However, at the first time of presentation, the localization of tumor was often difficult by CT or ultrasonography because the tumor was relatively too small. The introduction of endoscopic ultrasonography has allowed high-resolution imaging of the pancreas that distinguishes structures as small as 2 to 3 mm in diameter. Thus, it has became easier to detect the lesion site of pancreatic endocrine tumor by endoscopic ultrasonography. We report the 5 patients who had pancreatic insulinoma which were detected and localized by endoscopic ultrasonography.
Endosonography* ; Gastrinoma ; Humans ; Insulinoma* ; Pancreas ; Ultrasonography

Pancreatic endocrine tumors (PET) are rare neoplasms of the pancreas and account for less than 5% of all primary pancreatic malignancies. Included in this group are insulinomas, gastrinomas, glucagonomas and somatostatinomas. Collectively, these neoplasms are classified as functional PETs. When a PET is not associated with a clinical syndrome due to hormone oversecretion, it is referred to as a non-functional PET. Non-functionalPETs are pancreatic tumors with endocrine differentiation but lack a clinical syndrome of hormone hypersecretion. Although a pancreatic carcinoma shows aggressive biological behavior, a cutaneous metastasis from a pancreas carcinoma is rare. We report a case of a case of a cutaneous metastasis from an endocrine pancreatic carcinoma in a 50-year-old female that clinically manifested as a painful firm nodule on the back.
Female ; Gastrinoma ; Glucagonoma ; Humans ; Insulinoma ; Middle Aged ; Neoplasm Metastasis ; Pancreas ; Pancreatic Neoplasms ; Somatostatinoma

In patient with Zollinger-Ellison syndrome, it is difficult to localize gastrinoma because the tumor is frequently small and multiple. However, accurate localization of the tumor is important for the treatment. Among various imaging modalities, somatostatin receptor scintigraphy (SRS) has been recognized to be the most sensitive tool for the detection of neuroendocrine tumors such as gastrinomas based on the presence of high-affinity binding sites for somatostatin. Recently, we experienced a case of Zollinger-Ellison syndrome caused by gastrinomas which was localized by SRS. This is the first case report of gastrinoma detected by SRS in Korea. SRS can facilitate tumor detection in patient with Zollinger-Ellison syndrome and should be considered as the first-line diagnostic method in the early course of the disease.
Binding Sites ; Gastrinoma* ; Humans ; Korea ; Neuroendocrine Tumors ; Radionuclide Imaging ; Receptors, Somatostatin ; Somatostatin ; Zollinger-Ellison Syndrome*

BACKGROUND: Neuroendocrine tumors of the pancreas are classified according to the endocrine function as insulinomas gastrinomas somatostatinomas, or nonfunctioning tumors. However, the morphologic features are not different from each other. Therefore, we tried to compare correlations among the morphologic features, endocrine function, and the immunohistochemical reaction with specific monoclonal antibodies to the tumors. METHOD: We reviewed the medical records of seven patients with pancreatic neuroendocrine tumors retrospectively, and analysed the clinical manifestations, the methods of diagnosis, the pathological characteristics and the results of surgery. Additionally, we compared the correlation between the clinical manifestations and the expression of immunohistochemical staining by using six different kinds of monoclonal antibodies to each tumor. RESULTS: The seven pancreatic neuroendocrine tumor patients were treated by surgical excision. Four patients had benign insulinomas, two had nonfunctioning malignant tumor and one patient had a benign nonfunctioning tumor associated with stomach cancer. The pattern of immunohistochemical stain of each tumor was not correlate with the clinical manifestations. CONCLUSION: The morphologic study with H & E stain, even with immunohistochemical staining of pancreatic neuroendocrine tumor, cannot support differentiation of the functional diagnoses, such as insulinoma, gastrinoma, somatostatinoma, nonfunctional tumors and so forth.
Antibodies, Monoclonal ; Diagnosis ; Gastrinoma ; Humans ; Insulinoma ; Medical Records ; Neuroendocrine Tumors* ; Pancreas ; Retrospective Studies ; Somatostatinoma ; Stomach Neoplasms

Pancreatic endocrine tumors (PET) are rare neoplasms of the pancreas accounting for less than 5% of all primary pancreatic malignancies. Insulinomas, gastrinomas, glucagonomas and somatostatinomas is included in PET. PETs are usually classified into functioning and non-functioning tumors and presents with a range of benignity or malignancy. It is very important to accurate diagnose the PET location and to predict the benignity or malignancy of PET in terms of the treatment strategy, because PET have higher respectability, better response to chemotherapy and better prognosis compared to that of pancreatic adenocarcinoma. The utility and reliability of different imaging modalities depends on the characteristics of PETs, specifically their size. Functioning PET tend to be small (less than 2 cm), well circumscribed, homogeneous, and usually shown as strong enhancement on contrast enhanced CT or MR imaging. Non-functioning PET tend to be larger (4~10 cm), heterogeneous, and may contain the cystic areas of degeneration and necrosis. In this article, we present the various imaging findings of PET according to recent WHO classification.
Accounting ; Adenocarcinoma ; Gastrinoma ; Glucagonoma ; Insulinoma ; Magnetic Resonance Imaging ; Necrosis ; Pancreas ; Prognosis ; Somatostatinoma

A 34-year-old man was referred to our hospital with gastric polypoid lesions and biopsy-confirmed neuroendocrine tumor (NET). Computed tomography (CT) revealed a 3×3.5×8-cm retroperitoneal mass behind the pancreas, with multiple hepatic metastases. His serum gastrin level was elevated to 1,396 pg/mL. We performed a wedge resection of the stomach, a right hemi-hepatectomy, and a retroperitoneal mass excision. After careful review of the clinical, radiological, histopathological, and immunohistochemical findings, peripancreatic gastrinoma, and synchronous gastric NET were ultimately diagnosed. We reviewed a CT scan that had been performed 6 years previously after surgery for a duodenal perforation. There was no evidence of gastric or hepatic lesions, but the retroperitoneal mass was present at the same site. Had gastrinoma been detected earlier, our patient could have been cured using less invasive treatment. This case demonstrates how important it is to consider Zollinger-Ellison syndrome in patients with a recurrent or aggressive ulcer.
Adult ; Gastrinoma* ; Gastrins ; Humans ; Lymph Nodes* ; Neoplasm Metastasis ; Neuroendocrine Tumors* ; Pancreas ; Stomach ; Tomography, X-Ray Computed ; Ulcer ; Zollinger-Ellison Syndrome

The eradication of Helicobacter pylori and the widespread use of effective antisecretory therapies, including proton pump inhibitors, have improved the management of peptic ulcer disease. However, in some patients, peptic ulcer disease is refractory to 8 to 12 weeks of standard antisecretory drug treatment. For refractory peptic ulcer disease, further evaluation of the risk factors and causes of refractory peptic ulcer disease, including patient risk factors and noncompliance (smoking, nonsteroidal anti-inflammatory drug use, and noncompliance with medical treatment), persistent H. pylori infection, and non-H. pylori-related factors (giant ulcer, gastrinoma, infections other than H. pylori, and malignancy), is essential. The treatment should focus on the cause of the refractory peptic ulcer disease, avoiding smoking and nonsteroidal anti-inflammatory drug, the treatment of persistent H. pylori, use of high-dose proton pump inhibitors, or surgical excision of gastrinomas. Surgery should be considered in patients who are at high risk for complications and recurrent peptic ulcer disease despite medical treatment. In this review, I describe the diagnosis and treatment of refractory peptic ulcer disease.
Anti-Inflammatory Agents, Non-Steroidal ; Diagnosis ; Gastrinoma ; Helicobacter pylori ; Humans ; Peptic Ulcer ; Proton Pump Inhibitors ; Risk Factors ; Smoke ; Smoking ; Ulcer

Objective: To compare clinical characteristics of sporadic gastrinoma and multiple endocrine neoplasia type 1 (MEN1)-related gastrinoma. Methods: A retrospective cohort study was conducted. Patients with clinical manifestations of Zollinger-Ellison syndrome, pathological diagnosis as neuroendocrine neoplasm (NEN) and complete clinical and follow-up data were enrolled. Patients with only high gastric acid secretion but without evidence of NEN, or with other concurrent non-NEN tumors were excluded. According to the above criteria, the clinicopathological data of 52 cases of gastrinoma diagnosed from April 2003 to December 2020 in the First Affiliated Hospital, Sun Yat-sen University, were collected. Patients who met the diagnostic criteria of gastrinoma and met one of the following conditions were diagnosed as MEN1-related gastrinoma: (1) the presence of pathogenic mutations in the MEN1 gene confirmed by genetic testing; (2) NENs involving two or more endocrine glands, namely, pituitary, parathyroid, thymic, pancreatic, and adrenal NENs; (3) NEN and at least one first-degree relatives diagnosed as MEN1. The remaining gastrinomas were defined as sporadic gastrinoma. Student's t test and chi-square test were used for statistical analysis. Clinicopathological characteristics, endoscopic findings, imaging characteristics, treatment, and prognosis of sporadic and MEN1-related gastrinoma were compared. Results: Among 52 patients with gastrinoma, 33 were sporadic gastrinoma and 19 were MEN1-related gastrinoma. The common symptoms of both sporadic and MEN1-related gastrinomas were diarrhea (24/33, 72.7%; 17/19, 89.5%) and abdominal pain (19/33, 57.6%; 9/19, 47.4%). Compared with sporadic gastrinoma, MEN1-related gastrinoma needed longer time for diagnosis [(7.4±4.9) years vs. (3.9±5.2) years, t=-2.355, P=0.022), were more likely multiple tumors [47.4% (9/19) vs. 15.2% (5/33), χ(2)=6.361, P=0.012], had smaller diameter [(1.7±1.0) cm vs. (3.1±1.8) cm, t=2.942, P=0.005), presented the lower tumor grade [G1: 83.3% (15/18) vs. 39.4% (13/33); G2: 11.1% (2/18) vs. 54.5% (18/33); G3: 5.6% (1/18) vs. 6.1% (2/33), Z=-2.766, P=0.006], were less likely to have serum gastrin which was 10 times higher than normal [11.8% (2/17) vs. 56.0% (14/33), χ(2)=8.396, P=0.004], had higher probability of complication with type 2 gastric neuroendocrine tumors (g-NET) [31.6% (6/19) vs. 3.0%(1/33), χ(2)=6.163, P=0.013], and had lower rate of liver metastasis [21.1% (4/19) vs. 51.5% (17/33), χ(2)=4.648, P=0.031). There was no obvious difference between sporadic gastrinomas and MEN1-related gastrinomas in endoscopic findings. Both types presented enlarged and swollen gastric mucosa under the stimulation of high gastric acid, and multiple ulcers in the stomach and duodenum could be seen. Gastrinoma with type 2 g-NET presented multiple polypoid raised lesions in the fundus and body of the stomach. (68)Ga-SSR-PET/CT scan had a 100% detection rate for both types while (18)F-FDG-PET/CT scan had a higher detection rate for sporadic gastrinoma compared with MEN1-related gastrinoma [57.9% (11/19) vs. 20.0% (3/15), χ(2)=4.970, P=0.026]. Among the patients with sporadic gastrinoma, 19 received surgical treatment, 1 underwent endoscopic submucosal dissection, 8 underwent transcatheter arterial embolization (TAE), and 5 underwent surgery combined with TAE. Among patients with MEN1-related gastrinoma, 13 received surgical treatment, and the other 6 received conservative treatment. The median follow-up of all the patients was 21.5 (1-129) months, and the 5-year survival rate was 88.4%. The 5-year survival rate of patients with sporadic and MEN1-related gastrinomas was 89.5% and 80.0% respectively (P=0.949). The 5-year survival rate of patients with and without liver metastasis was 76.2% vs. 100%, respectively (P=0.061). Conclusions: Compared with sporadic gastrinoma, MEN1-related gastrinoma has longer diagnosis delay, smaller tumor diameter, lower tumor grading, lower risk of liver metastasis, and is more likely to complicate with type 2 g-NET, while there is no difference in survival between the two tumor types.
Gastrinoma/genetics* ; Humans ; Multiple Endocrine Neoplasia Type 1/genetics* ; Pancreatic Neoplasms/genetics* ; Positron Emission Tomography Computed Tomography ; Retrospective Studies