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)

One hundred and four consecutive cases of carcinoid among 194 APUD-omas (cerebral and gynecological tumours expected) are presented. Localization is as follows: bronchopulmonary (21%), duodenum (5,5%), small intestine (19%), Meckel's diverticulum (5,5%), appendix (39%), colorectal (5,5%), other (4,5%). Average age at diagnosis is very different for each localization of carcinoid tumour, as is prognosis: in our material, 33% of cases had an infiltrative tumour, 17% had lymph nodes metastases and 16% hepatic metastases. All 15 patients with carcinoidosis died but their survival varied considerably in length (from a few weeks to 21 years after the beginning of carcinoidosis). In addition, 3 patients presented with the Zollinger-Ellison syndrome, 2 of them having an atypical or probable form of the MEN I syndrome.
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PMID:[Carcinoid tumors, carcinoid syndromes and related tumors (104 cases)]. 720 84

We determined the relative concentrations of gastrin molecular species in serum samples from 21 patients with Zollinger-Ellison syndrome with localized gastrinoma (n = 11) or gastrinoma with hepatic metastases (n = 10). Gastrin molecular species were separated by gel-filtration chromatography and quantitated by radioimmunoassay with a gastrin antiserum produced in our laboratory. The percentage gastrin-17 of the total gastrin in the two groups differed significantly (nonparametric Wilcoxon rank test; p less than 0.01). Patients with the Zollinger-Ellison syndrome with apparently localized gastrinoma had a lower percentage of G- 17 (7.6%, SEM 1.6%) than did patients with gastrinoma with hepatic metastases (31.1%, SEM 6.1%). This procedure may be useful in the early classification of tumors in patients with Zollinger-Ellison syndrome.
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PMID:Serum gastrins in Zollinger-Ellison syndrome: identification of localized disease. 737 8

Prior to total gastrectomy, serum levels of gastrin and human chorionic gonadotropin (HCG) and its alpha- and beta-subunits (alpha-HCG and beta-HCG) were determined by radioimmunoassays in 40 patients with the Zollinger-Ellison syndrome. Basal serum gastrin levels greater than 1,500 pg/mL were found only in patients with metastases to lymph nodes or liver, while levels greater than 8,000 indicated massive liver replacement by tumor. Gastrin levels less than 1,500 pg/mL had no correlation with malignant behavior. Neither the calcium-infusion nor secretin-injection test was useful in identifying tumors as benign or malignant. Basal serum levels of alpha-HCG were elevated (> 7 ng/mL) in four of 20 patients with metastatic gastrinoma and were normal in all 16 patients with benign disease. There was a significant correlation between basal gastrin and alpha-HCG levels in patients with malignant gastrinoma but not for those with benign tumors. The results suggest that serum gastrin and alpha-HCG levels can be useful in assessing the biologic behavior of gastrinomas and in planning appropriate surgical and nonsurgical treatment.
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PMID:Serum gastrin and human chorionic gonadotropin in the Zollinger-Ellison syndrome. 741 55

Among endocrine tumors arising in the intestinal tract, midgut argentaffin EC cell carcinoids, duodenal gastrin cell tumors and rectal trabecular L cell carcinoids, in order of decreasing frequency, are those better represented. Together they account for more than 80% of such tumors. Duodenal somatostatin cell tumors, gangliocytic paragangliomas and poorly differentiated neuroendocrine carcinomas, are also well defined tumor entities. The carcinoid syndrome with intermittent flushing, hypotension and diarrhea, and the Zollinger-Ellison syndrome with severe peptic ulcer disease, are the only hyperfunctional syndromes consistently found in association with these tumors. The carcinoid syndrome arises in about 10% of intestinal carcinoids, usually in their advanced metastatic stage. The Zollinger-Ellison syndrome occurs in association with about 40% of gastrin cell tumors, including small intramural growths. Tumor prognosis depends on mode and site of presentation, histology, cell type(s), size, level of invasion, metastases (especially distant metastases) and associated clinical syndrome or background disease.
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PMID:Endocrine tumors of the small and large intestine. 747 53

The surgical treatment of patients with the Zollinger-Ellison syndrome has undergone a dramatic evolution since the syndrome was originally described. It is now recognized that an aggressive surgical approach is mandatory because of the malignant potential of gastrinomas in both the sporadic and the familial forms of the syndrome. Although initially regarded as an incurable neoplasm, it is now known that complete surgical resection of gastrinomas can result in eugastrinemia even in the presence of lymph node metastases. It is now recognized that extrapancreatic gastrinomas are more common than pancreatic gastrinomas, and the most common location for an extrapancreatic gastrinoma is the duodenal wall. Major improvements in preoperative imaging and intraoperative localization techniques combined with an increased awareness of the anatomic distribution of gastrinomas have markedly increased the surgeon's ability to care for and cure patients with the Zollinger-Ellison syndrome.
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PMID:The place for curative surgical procedures in the treatment of sporadic and familial Zollinger-Ellison syndrome. 758 13

Zollinger-Ellison syndrome (ZES) is caused by gastrin-secreting tumors called gastrinomas. Patients commonly present with peptic ulcer disease and may have recurrent, multiple, and atypically located ulcers, e.g. in the jejunum. Alternatively, severe diarrhea may be the only presenting symptom. Patients with multiple endocrine neoplasia Type I (MEN-I) and ZES become symptomatic at an earlier age than patients with sporadic ZES. Patients with ZES have elevated fasting serum gastrin concentrations (> 100 pg/ml) and basal gastric acid hypersecretion (> 15 mEq/h). The secretin stimulation test is the best test to distinguish ZES from other conditions resulting in elevated gastrin levels. Gastric acid hypersecretion can be controlled in virtually all patients with H2-receptor antagonists or omeprazole, thus rendering total gastrectomy unnecessary. Computed tomography (CT), magnetic resonance imaging (MRI), radionuclide octreotide scanning, endoscopic ultrasound, and the selective arterial secretin injection test are the recommended imaging studies for localization of gastrinoma; nevertheless, 50% of gastrinomas are not evident on preoperative imaging studies. All patients with sporadic gastrinoma who do not have unresectable metastatic disease should undergo exploratory laparotomy for potential curative resection. With increased awareness of duodenal tumors, gastrinoma can be found in 80-90% of patients. Surgery may be the most effective treatment for metastatic gastrinoma if most or all of the tumor can be resected. The management of patients with MEN-I and ZES remains controversial. Some clinicians advocate an aggressive surgical approach, whereas others have had little success in rendering patients eugastrinemic.
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PMID:Management of patients with Zollinger-Ellison syndrome. 759 74

Between 1987 and 1991, 16 patients (13 females, three males; mean age 52.4 [33-73] years) with Zollinger-Ellison syndrome (ZES) were treated according to a standardized surgical concept. The diagnostic work-up consisted of measuring serum gastrin levels, pre-operative localization by ultrasound and abdominal computed tomography, as well as extensive staging by laparotomy. As complete a tumour resection as possible was the aim of treatment in 15 patients, while in one patient it was to reduce the tumour mass. In six patients who had resection of a solitary tumour there was no evidence of recurrence after 6-42 months of follow-up. Exploration of the duodenum made it possible to identify and then remove a small gastrinoma of the duodenal wall in three patients. No gastrinoma was found in one patient despite extensive exploration. In a further four patients the laparotomy was purely exploratory, because diffuse metastasization was found. In four patients the primary tumour and, where present, the regional lymph nodes were removed, but the signs of ZES persisted, i.e. the intervention was merely palliative. In one female patient, reduction of tumour mass was necessary because the symptoms could not be controlled by conservative measures: she died postoperatively from toxic hepatitis. This experience indicates that standardized surgical intervention achieves potentially curative results in nearly 40% of patients. Including the duodenum in the surgical exploration allows the identification of even small gastrinomas of the duodenal wall. It is concluded that all patients with ZES but no evidence of diffuse metastases should undergo surgical treatment.
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PMID:[Standardized surgical concept for the diagnosis and therapy of Zollinger-Ellison syndrome]. 768 72

Four types of gastric carcinoids have been identified: (1) multiple small body-fundus carcinoids associated with chronic atrophic gastritis type A (A-CAG); (2) sporadic solitary lesions without specific pathogenetic background (non-A-CAG); (3) carcinoidosis associated with Zollinger-Ellison/MEN 1 syndrome, and (4) rare tumors, e.g. gastrin cell tumors, neuroendocrine carcinomas and mixed endocrine-exocrine tumors. In a retrospective study of 15 patients with gastric carcinoids (11 A-CAG, 3 non-A-CAG and 1 gastrin cell tumor) over a 10-year period, the histopathological and clinical features were assessed. The A-CAG-type carcinoids were clinically silent with lymph node metastases in 2/11 cases but no hepatic metastases. The non-A-CAG-type carcinoids were malignant with disseminated disease, hormonal symptoms and increased urinary excretion of the main histamine metabolite, MeImAA. Five patients with A-CAG tumors were subjected to antrectomy to remove hypergastrinemia, which is thought to be of pathogenetic importance for these tumors. During the observation period (1.5-8 years) 1 patient developed recurrent tumors, while the other 4 showed persistent argyrophil cell hyperplasia. A prospective treatment protocol of these tumors is suggested with endoscopic removal of less numerous, small lesions as first-step therapy, followed by antrectomy at recurrence. Larger lesions should be excised in combination with antrectomy. Gastrectomy is reserved for the rare cases of invasive tumors with lymph node metastases. As evident from the outcome of patients with non-A-CAG tumors radical surgery should be performed whenever practicable.
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PMID:Clinical management of gastric carcinoid tumors. 769 42

A detailed description of recent advances in the management of patients with Zollinger-Ellison syndrome (ZES) is presented. The clinical presentation is reviewed, and newer diagnostic tools, both preoperative and intraoperative, are discussed. An update on surgical management is presented, including indications for abdominal exploration, intraoperative localization techniques, surgical excision, and the approach to patients with metastases or in whom no tumor is found. New strategies in the medical management of ZES also are reviewed. An update on patient survival is presented, and a review of the management of patients with multiple endocrine neoplasia type 1 and ZES is discussed.
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PMID:Advances in the management of patients with Zollinger-Ellison syndrome. 774 56

Neuroendocrine gut and pancreatic tumors are neoplasms that present distinct features from other malignant tumors. Firstly, in most patients, tumor growth is rather slow, and even in advanced metastatic disease, there is very little impairment of the general well-being of the individual, e.g. appetite and weight. Secondly, these tumors are known to produce specific peptide hormones which may be factors in some clinical conditions e.g. carcinoid, Zollinger-Ellison and hypoglycemic syndromes. These conditions can be critical to the patients and can occasionally be lethal. Therefore, the treatment of neuroendocrine tumors must control the clinical symptoms related to hormone over-production and prevent further tumor growth. These two features are not always in parallel. Systemic treatment of neuroendocrine tumors mainly consists of chemotherapy, interferon and somatostatin analog administration. Chemotherapy has been used for at least 30 years; the most effective combination has proved to be streptozotocin with 5-fluorouracil or adriamycin. This combination produces biochemical responses in up to 60% of patients with endocrine pancreatic tumors; the results in carcinoid patients are very poor and response rates are < or = 10%. Alpha-interferon (IFN-alpha) produces biochemical responses in approximately 50% of patients with malignant carcinoid tumors, significant reductions in tumor size in 15% and a further 39% of patients have disease stabilization with no further tumor growth. Somatostatin analogs have only been used clinically within the last 10 years, but produce symptomatic improvement in 70% of cases, biochemical responses in 40-60%, but rarely produce any significant reduction in tumor size. These analogs are particularly useful to control severe clinical symptoms and are the first-line therapy for the management of carcinoid patients both peri- and intra-operatively. Patients with endocrine pancreatic tumors, particularly those with glucagon and vasointestinal peptide-producing tumors, benefit most from this type of treatment. Recently, a combination of IFN-alpha and a somatostatin analog has showed an additive effect of these two drugs. The side effects of streptozotocin and 5-fluorouracil are mainly nausea and vomiting which can be controlled with 5-HT3 receptor blocker therapy. Another significant adverse reaction is impaired renal function. The adverse reactions to IFN-alpha are mainly flu-like symptoms, fatigue, mild impairment of liver and bone marrow function and autoimmune reactions in 15% cases. Somatostatin analog treatment causes a low frequency of adverse reactions, those which do occur include gall stone formation and steatorrhea. Future systemic treatment should be based on increased knowledge of the tumor biology, particularly growth-regulatory mechanisms.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Endocrine tumors of the gastrointestinal tract: systemic treatment. 785 82


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