Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Although gastrinoma resection is generally advocated for patients with the sporadic form of nonmetastatic Zollinger-Ellison syndrome, there is controversy regarding the surgical management of the gastrinoma among patients with multiple endocrine neoplasia type I (MEN-I). Using strict criteria, to date no biochemical cures of the Zollinger-Ellison syndrome lasting greater than 5 months have been achieved by gastrinoma resection among patients with MEN-I. Whereas resections of hepatic metastases have been performed in patients with sporadic gastrinoma, none have been reported among patients with MEN-I. The current report describes a patient with MEN-I, closely followed up for 30 years, in whom enlargement of pancreatic gastrinoma and development of hepatic gastrinoma was observed to occur over 3 years. After preoperative localization, an 80% pancreatectomy and a left lateral segmentectomy of the liver were performed. Sixteen months after the operation, secretin and calcium provocative testing showed that the patient's fasting gastrin and stimulated plasma gastrin concentrations were normal; also, results of computerized tomographic angiography, selective abdominal angiography, and hepatic venous sampling for gastrin after intra-arterial secretin injection were negative for gastrinoma. By achieving a 16-month cure of gastrinoma, this case shows that an aggressive surgical approach can benefit certain patients with gastrinoma who have MEN-I even in the presence of hepatic metastases.
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PMID:Benefit of resection of metastatic gastrinoma in multiple endocrine neoplasia type I. 134 27

Before the mid-1970s, the most reliable therapy for Zollinger-Ellison syndrome (Z-E syndrome) was total gastrectomy since complete excision of gastrinoma is difficult in many cases due to multifocus and/or metastases. With the advent of H2-blockers gastric acid secretion can be controlled at safe levels (less than 10 mEq/h), and patients with Zollinger-Ellison syndrome can be managed medically. The problem of therapy with H2-blockers however, is that large doses are usually required and that a marked tachyphylaxis is frequently noted during long-term treatment. Treatment with new antisecretory drug, proton pump inhibitor, has been shown to be highly effective with relatively high doses and without tachyphylaxis, although long-term experience is still limited.
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PMID:[Application of proton pump inhibitor to special conditions; Zollinger-Ellison syndrome]. 134 27

A retrospective study of 90 surgically treated patients with the Zollinger-Ellison syndrome seen from 1958 through 1990 was performed. Fifteen patients had Zollinger-Ellison syndrome as a manifestation of multiple endocrine neoplasia type I. Preoperative tumor localization was positive in 46% of 54 patients studied. Gastrinomas were identified in 66% of patients, 38% of the tumors being malignant. Postoperative eugastrinemia was achieved in 11% of patients after a variety of surgical procedures. Exploratory laparotomy provides the only chance for cure and identifies the significant prognostic factors associated with long-term patient survival: small tumor size, extrapancreatic primary, and absence of tumor metastases.
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PMID:The Zollinger-Ellison syndrome. A collective surgical experience. 135 63

Islet cell neoplasia is a frequent occurrence in multiple endocrine neoplasia type 1 (MEN 1). Sixteen of 27 patients with MEN 1 developed functioning endocrine pancreatic tumor syndromes. Eleven of the 16 developed Zollinger-Ellison syndrome and each was evaluated by a combination of computed tomography and hepatic angiography to exclude hepatic metastasis and percutaneous transhepatic catheterization to localize the tumor. Seven of the 11 patients were found to have duodenal gastrinomas with multiple duodenal tumors in three patients. Four of the 11 patients had only pancreatic gastrinomas. In addition to the gastrinomas, other types of islet tumors in the pancreatic body or tail were found in nine of the 11 patients. None of the patients had hepatic metastases. Seven of the 11 patients were treated by distal pancreatectomy and since 1986 all patients have had duodenotomies as part of the surgical exploration. Postsurgical evaluation ranging from three months to 14 years indicates that 10 of 11 patients have normal basal gastrin levels. We conclude that duodenal gastrinomas are common in MEN 1 and can be managed successfully by appropriate operative intervention.
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PMID:Surgical treatment of the endocrine pancreas and Zollinger-Ellison syndrome in the MEN 1 syndrome. 136 4

Since 1980, 73 patients with Zollinger-Ellison syndrome (ZES) without radiographic evidence of liver metastases were studied on a prospective protocol including medical management of gastric acid hypersecretion, extensive radiographic tumor localization, and exploratory surgery to find and resect gastrinoma for potential cure. Each patient had gastric acid hypersecretion effectively controlled with either H2-blockers or omeprazole. Patients were divided prospectively into two groups, with all patients undergoing the same preoperative localization studies and extensive laparotomy. In contrast to group 1 (1980-1986) (36 patients), group 2 (1987-Oct. 1990) (37 patients) also underwent additional procedures (transillumination and duodenotomy) at surgery to find duodenal gastrinomas. Preoperative imaging studies localized tumor in 38 (52%) patients, and portal venous sampling for gastrin determinations was positive in 49 (67%) patients. Gastrinomas were found and resected in 57 (78%) patients. Significantly more gastrinomas (92% of patients) were found in group 2 than in group 1 patients (64%) (p less than 0.01). This increase was due to increased numbers of duodenal gastrinomas in group 2 than in group 1 patients (43% versus 11%; p less than 0.01). The increased ability to find duodenal gastrinomas did not significantly improve the immediate disease-free rate, which was 58% for all patients. Duodenal primary gastrinomas were found to have a significantly greater incidence of metastases (55%) and a significantly shorter disease-free interval (12 months) than pancreatic gastrinomas (22% and 84 months, respectively) suggesting that duodenal gastrinomas may be more malignant and not more frequently curable than pancreatic gastrinomas. Operations were performed with no deaths and 11% morbidity rate. Long-term follow-up showed that 50% of patients initially rendered disease free would develop recurrent disease by 5 years. Survival was excellent for all patients, and none died of malignant spread of the tumor or uncontrolled peptic ulcer disease, with a mean follow-up of 5 years. This finding is in contrast to patients who presented with metastatic disease on imaging studies and had a 20% 5-year survival rate. This study suggests that all patients with localized sporadic ZES can have the gastric acid hypersecretion managed medically, that overall survival of these patients is excellent, most (78%) can have all gastrinoma found and resected, and some (30%) will be cured (long-term disease-free survival).
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PMID:Curative resection in Zollinger-Ellison syndrome. Results of a 10-year prospective study. 153 Oct 4

The clinical features, diagnostic methods, management and survival in 16 cases of Zollinger-Ellison syndrome encountered in Northern Ireland between the years 1970 and 1988 are described. While the majority of patients in the first decade of the study period had surgical treatment, those presenting in the latter period have been managed with medical therapy in the form of H2-receptor antagonists or omeprazole. The increasing use of these agents seems to be altering the severe clinical features of this condition, reducing the indications for surgery and maintaining patients, with or without evidence of metastatic disease, in clinical remission.
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PMID:Changing patterns in presentation and management of the Zollinger-Ellison syndrome in Northern Ireland, 1970-1988. 167 70

A diagnostic and therapeutic strategy for ZES is proposed based on the vast experience (180 cases) of this rare disease gained at hospital Bichat (Paris, France). The first step is diagnostic and relies essentially a) upon measurements of gastric acid and serum gastrin in the basal state and b) upon the results of secretin test because of the overlap between ZES and duodenal ulcer disease in a large proportion of cases. In sporadic ZES cases, after adequate control of acid overproduction and attempt to localize the tumoral process(es), surgery is indicated (at the exception of patients with advanced metastatic disease). Surgery aims essentially at eradicating gastrinoma(s) as often as possible and whenever it is feasible without endangering patient's life. An apparently definite cure is attained in 60 to 80% in extrapancreatic gastrinoma(s) and 20 to 30% when gastrinomas are located within the pancreas. Liver involvement (25% of ZES cases) remains the major concern and death cause in these patients. Although liver metastases frequently stabilize and sometimes regress upon chemotherapy and chemoembolization, liver transplantation may, in the future, represent the major chance for these patients; but in this frequently slowly evolving condition, the time for liver transplantation is exceptionally difficult to settle.
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PMID:[Which diagnostic and therapeutic approach to the Zollinger-Ellison syndrome should be adopted in 1990?]. 167 64

Zollinger-Ellison syndrome (ZE) is characterized by severe peptic ulcer disease, hypersecretion of gastric acid and gastrinoma of the pancreas. A 56-year-old woman with abdominal pain, vomiting, diarrhea, and weight loss with ZE is presented. Large doses of H2 blockers were ineffective. At operation hepatic metastases from a gastrinoma were found and highly selective vagotomy (HSV) was performed. The combination of omeprazol and streptozotocin then lowered gastric secretion to normal. Conservative operation has included HSV or total gastrectomy. Medical treatment based on H2 blockers or omeprazol and chemotherapy are given as necessary.
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PMID:[Treatment of Zollinger-Ellison syndrome]. 167 28

Ninety-nine carcinoid tumors of the duodenum were studied. Seventy-seven patients were followed up for a mean period of 65 months, 20 tumors were autopsy findings, and two patients were unavailable for follow-up. Sixteen tumors (21%) produced metastases, all discovered initially; 3 patients (4%) died from metastatic disease (mean survival, 37 months postoperatively). Features associated with metastatic risk were involvement of muscularis propria, size greater than 2 cm, and the presence of mitotic figures. For 51 tumors, there was no correlation between immunohistochemical somatostatin and history of diarrhea, cholelithiasis, or diabetes mellitus (somatostatin syndrome). Five tumors were associated with Zollinger-Ellison syndrome and had immunohistochemical gastrin, but in the others there was no correlation between ulcer disease and gastrin positivity. Duodenal carcinoids are indolent, especially when small and localized to the submucosa. Immunohistochemical identification of somatostatin and gastrin has little clinical relevance.
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PMID:Carcinoid tumors of the duodenum. A clinicopathologic study of 99 cases. 169 55

In patients with multiple endocrine neoplasia type 1 (MEN-1), gastrinomas are common and thought to occur predominantly in the pancreas. We describe eight patients with MEN-1 and hypergastrinemia (seven with the Zollinger-Ellison syndrome) in whom we searched for neuroendocrine tumors in the pancreas and duodenum. Tumors were found in the proximal duodenum in all eight patients: solitary tumors (diameter, 6 to 20 mm) in three patients and multiple microtumors (diameter, 2 to 6 mm) in the other five. Paraduodenal lymph-node metastases were detected in four patients. Immunocytochemical analysis revealed the presence of gastrin in all the duodenal tumors and in their lymph-node metastases. In contrast, no immunoreactivity for gastrin was present in the endocrine tumors found in the seven pancreatic specimens available for study, except for one tumor with scattered gastrin-positive cells. In four of the six patients whose duodenal gastrinomas were removed, serum gastrin levels returned to normal; in the other two patients gastrin concentrations decreased toward normal. We conclude that in patients with MEN-1 and the Zollinger-Ellison syndrome, gastrinomas occur in the duodenum, but the tumors may be so small that they escape detection.
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PMID:Gastrinomas in the duodenums of patients with multiple endocrine neoplasia type 1 and the Zollinger-Ellison syndrome. 197 64


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