Primary hepatic gastrinoma is a rare disease, with fewer than 40 cases reported in the medical literature. Because it is located in an organ in which metastases are common, its diagnosis is difficult. We report a case of a 19 years old male patient with a history of gastric ulcers since the age of nine. Following gastric surgery, an antrectomy and a vagotomy, there was some alleviation of symptoms. Subsequently, the patient reported various intermittent episodes of diarrhea, diffuse abdominal pain, and vomiting. The patient underwent tomography, which revealed the presence of a hepatic mass measuring 19.5 cm × 12.5 cm × 17 cm. Primary hepatic gastrinoma was diagnosed based on laboratory examinations that indicated hypergastrinemia and a positron emission tomography/magnetic resonance study with somatostatin analogue that confirmed the liver as the primary site. After hepatic trisegmentectomy(Ⅱ, Ⅲ, Ⅳ, Ⅴ, Ⅷ), the patient's symptoms improved. The case is notable for the presence of a rare tumor with uncommon dimensions. 相似文献
Gastrinomas, a rare group of neuroendocrine tumors, are responsible for severe peptic disease and diarrhea. Although symptomatic control may be achieved with proton‐pump inhibitors (PPIs) and somatostatin analogues (SSAs), data are limited regarding the possible antitumor effect of the peptide receptor radioligand therapy (PRRT) with radiolabeled SSAs in gastrinoma patients. The goal of this study was to assess the effect of PRRT on symptoms, gastrin secretion, and tumor load in patients with progressive malignant gastrinomas.
METHODS:
We retrospectively studied 11 patients with metastatic gastrinomas followed for a mean period of 6 years. All patients were symptomatically treated with PPIs, and 9 of 11 patients received monthly injections of SSAs; all patients had an Eastern Cooperative Oncology Group score of 0‐1, and received PRRT (90Yttrium‐ or 177Lutetium‐DOTATOC) for progressive disease. Serum gastrin measurements and radiological assessment (using the Response Evaluation Criteria in Solid Tumors criteria) were performed before and every 3‐6 months following PRRT.
RESULTS:
PRRT induced symptomatic improvement in all patients. The mean serum gastrin decreased significantly from 4831 mI/L to 932.6 mI/L (normal, 40‐108 mI/L; P < .001). Periodic radiological surveillance showed complete response in 1 (9%) patient, partial tumor response in 5/11 (45%) patients, and tumor stabilization in 5/11 (45%) patients. In 7/11 (64%) patients, the antitumor effect of PRRT persisted after a median period of 14 months. Four of 11 (36%) patients died due to tumor progression (median time to progression, 11 months); in this group, the mean survival time after the last PRRT was 14 ± 6.9 months.
AIM:To describe our patients affected with ectopic biliary tree gastrinoma and review the literature on this topic.METHODS:Between January 1992 and June 2012,28 patients affected by duodenopancreatic endocrine tumors in multiple endocrine neoplasia type 1(MEN1)syndrome underwent surgery at our institution.This retrospective review article analyzes our experience regarding seventeen of these patients subjected to duodenopancreatic surgery for Zollinger-Ellison syndrome(ZES).Surgical treatment consisted of duodenopancreatectomy(DP)or total pancreatectomy(TP).Regional lymphadenectomy was always performed.Any hepatic tumoral lesions found were removed during surgery.In MEN1 patients,removal of duodenal lesions can sometimes lead to persistence or recurrence of hypergastrinemia.One possible explanation for this unfavorable outcome could be unrecognized ectopic localization of gastrin-secreting tumors.This study described three cases among the seventeen patients who were found to have an ectopic gastrinoma located in the biliary tree.RESULTS:Seventeen MEN1 patients affected with ZES were analyzed.The mean age was 40 years.Fifteen patients underwent DP and two TP.On histopathological examination,duodeno pancreatic endocrine tumors were found in all 17 patients.Eighty-one gastrinomas were detected in the first three portions of the duodenum.Only one gastrinoma was found in the pancreas.The mean number of gastrinomas per patient was 5(range 1-16).Malignancy was established in 12 patients(70.5%)after lymph node,liver and omental metastases were found.Three patients exhibited biliary tree gastrinomas as well as duodenal gastrinoma(s).In two cases,the ectopic gastrinoma was removed at the same time as pancreatic surgery,while in the third case,the biliary tree gastrinoma was resected one year after DP because of recurrence of ZES.CONCLUSION:These findings suggest the importance of checking for the presence of ectopic gastrinomas in the biliary tree in MEN1 patients undergoing ZES surgery. 相似文献
A primary hepatic gastrinoma found in a 13-year-old boy was studied by light microscopy, immunohistochemistry, electron microscopy, and immunoelectron microscopy. Results were consistent with a neuroendocrine neoplasm with abundant gastrin-immunoreactive cells. Unlike all previously reported cases of primary hepatic neuroendocrine tumors, which have been endocrinologically asymptomatic, the patient had a Zollinger-Ellison syndrome apparently cured by surgical resection of the tumor. 相似文献
Introduction: Recently, there have been a number of advances in imaging pancreatic neuroendocrine tumors (panNETs), as well as other neuroendocrine tumors (NETs), which have had a profound effect on the management and treatment of these patients, but in some cases are also associated with controversies.
Areas covered: These advances are the result of numerous studies attempting to better define the roles of both cross-sectional imaging, endoscopic ultrasound, with or without fine-needle aspiration, and molecular imaging in both sporadic and inherited panNET syndromes; the increased attempt to develop imaging parameters that correlate with tumor classification or have prognostic value; the rapidly increasing use of molecular imaging in these tumors and the attempt to develop imaging parameters that correlate with treatment/outcome results. Each of these areas and the associated controversies are reviewed.
Expert commentary: There have been numerous advances in all aspects of the imaging of panNETs, as well as other NETs, in the last few years. The advances are leading to expanded roles of imaging in the management of these patients and the results being seen in panNETs/GI-NETs with these newer techniques are already being used in more common tumors. 相似文献
Pancreatic neuroendocrine tumours are uncommon neoplasms of the pancreas, accounting for around 2% of all primary pancreatic tumors. This specific group of tumours includes insulinoma, gastrinoma, glucagonoma, VIPoma, somatostatinoma and others. They may cause a clinical syndrome due to hormone overproduction. These tumours tend to be less aggressive than pancreatic adenocarcinoma, however, more than 50% of them have metastasized to the liver at the time of diagnosis. Under this circumstance, surgery is impossible to resect all metastases. Thus other various medical measures have been explored in the treatment of these tumors. 相似文献
Palliative care (PC) seeks to improve the quality of life for patients facing serious illness. Several oncology organizations have guidelines about PC, reflecting the need to integrate PC into standard oncology care. Many surgical patients do not receive PC despite the need for these services and established surgical PC competencies. Recent educational and research efforts reveal increased appreciation of the need and benefit of integrating PC services with cancer care in general, and surgical care, specifically. 相似文献
We examined the characteristics of patients with Zollinger-Ellison syndrome who developed a perforation prior to diagnosis to determine whether any clinical features were useful markers of the syndrome. Of 160 patients with Zollinger-Ellison syndrome, perforation occurred prior to the diagnosis being made in 11 (7%). At surgery, perforations were found in the duodenum in six cases and in the jejunum in five. In no case was tumor identified at emergency surgery, and the diagnosis of Zollinger-Ellison syndrome was made only in the postoperative period when excessive gastric secretions were noted. Neither acid output nor serum gastrin concentration were useful predictors for perforation. The patients, six men and five women, were 27–61 years old (median 48) and one had MEN-1. Three patients had no symptoms prior to the perforation. The other eight had symptoms for 1–15 years, with diarrhea occurring in 45% of the cases. Following the diagnosis of Zollinger-Ellison syndrome, patients were given medication to control gastric acid hypersecretion. Eight patients remained well, but the three patients who had had a partial gastrectomy had a complicated course despite medical therapy. Although features of perforation in Zollinger-Ellison syndrome are not specific, jejunal perforation or perforation associated with a history of diarrhea is suggestive of the diagnosis. Serum gastrin should be measured in every case and a partial gastrectomy avoided. 相似文献
Multiple endocrine neoplasia type 1 (MEN1) gastro-entero-pancreatic (GEP) tumours develop from the pancreatic islets and from the endocrine cells of the duodenal and gastric mucosa. Even if GEP tumours have generally a benign course, a subgroup of them shows an aggressive behaviour and is a major cause of death amongst MEN1 patients. Diagnosis of insulinoma should lead promptly to pancreatic surgery. MEN1 gastrinomas are multiple and almost exclusively localized in the duodenum. Cure rate for Zollinger-Ellison syndrome in MEN1 is low when surgery is limited to tumour enucleation or full thickness duodenal wall resection. Conversely, pancreatoduodenectomy is followed by higher chance of cure. For nonfunctioning tumours exceeding 1 cm diameter in size a prompt treatment is recommended due to their high malignant potential. Gastroscopic surveillance is indicated for the frequent occurrence of multiple, small, type 2 fundic carcinoids. Endoscopic removal is possible for lesions growing in the mucosa-submucosa, but partial or even total gastrectomy is recommended for the small number of gastric carcinoids infiltrating the muscular layers. 相似文献