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)

In 1982, a prospective study was initiated of 52 consecutive patients with proven Zollinger-Ellison syndrome (ZES), involving surgical exploration with the goal of removing the gastrinoma after an extensive protocol to localize the tumor. Each patient underwent ultrasound, computed tomography (CT) with oral/intravenous (IV) contrast, and selective arteriography. Eighteen patients had metastatic disease identified by imaging studies and confirmed by percutaneous biopsies, and two patients had multiple endocrine neoplasia type I (MEN-I) with negative imaging studies; therefore, these 20 patients did not undergo laparotomy. Each of the remaining 32 patients (3 with MEN-I and positive imaging studies) underwent laparotomy, and gastrinomas were removed in 20 patients. Preoperative ultrasound localized tumors in 20% of patients, CT in 40%, arteriography in 60%, and any of the modalities in 70% of patients. Infusion CT and arteriography were 100% specific. In 18 patients with either negative imaging (17) or false-positive imaging (1 ultrasound), gastrinomas were found and removed in six patients (33%). Twenty-four gastrinomas were found in 20 patients at laparotomy: eight in lymph nodes around the pancreatic head, four in the pancreatic head, one in the pancreatic body, three in the pancreatic tail, three in the pyloric channel, one in the duodenal wall, two in the jejunum at the ligament of Treitz, one in the ovary, and multiple liver metastases in one patient. If one excludes patients with MEN-I or liver metastatic disease, 12/28 (43%) of patients were biochemically "cured" immediately after operation. This result decreased to 7/23 (30%) with greater than 6 months follow-up. No patients with gastrinomas resected have developed recurrent gastrinoma on follow-up imaging studies (longest follow-up: 4 years). This study indicates that 95% of metastatic gastrinoma can be diagnosed before operation and that, by a combination of careful imaging studies and thorough exploration at surgery, 30% of patients with gastrinomas may be curable.
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PMID:Prospective study of gastrinoma localization and resection in patients with Zollinger-Ellison syndrome. 353 71

The ability of routine computed tomography (CT) performed with oral and intravenous contrast to localize gastrinomas in 61 consecutive patients with Zollinger-Ellison syndrome was evaluated prospectively. The results of CT scanning were subsequently evaluated in all patients by either surgery, autopsy, or percutaneous biopsy. Thirteen of 14 patients with CT scans positive for hepatic metastases and 5 of 13 patients with CT scans negative for hepatic metastases were found to have gastrinoma in the liver. For gastrinoma metastatic to the liver, CT scanning had a specificity of 98%, a sensitivity of 72%, a positive predictive value of 93%, and a negative predictive value of 90%. Twenty-two of 23 patients with positive extrahepatic CT scans and 15 of 33 patients with negative extrahepatic CT scans were found to have extrahepatic gastrinomas. For extrahepatic gastrinoma, CT scanning had a specificity of 95%, a sensitivity of 59%, a positive predictive value of 96%, and a negative predictive value of 54%. The ability of CT scan to detect gastrinomas both in the liver and extrahepatically was directly related to tumor size, detecting 0% of tumors less than 1 cm and 83%-95% of tumors greater than 3 cm. The location of the extrahepatic gastrinoma was also an important determinant in that approximately 80% of pancreatic gastrinomas but only 35% of extrapancreatic gastrinomas were detected. The present results indicate that because of its convenience and accuracy, CT scanning with oral and intravenous contrast material should be the initial procedure to evaluate the extent of gastrinoma. A positive CT scan is almost always correct; therefore, a CT scan detecting metastatic gastrinoma to the liver would avoid unnecessary surgery and, if positive for extrahepatic gastrinoma, would assist the surgeon in finding the gastrinoma. A negative CT is less reliable; therefore, patients should undergo other localizing studies before exploratory laparotomy.
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PMID:Prospective study of the ability of computed axial tomography to localize gastrinomas in patients with Zollinger-Ellison syndrome. 355 96

Five patients with the Zollinger-Ellison syndrome who had extra-pancreatic (duodenal or paraduodenal) gastrinoma with lymph node metastases responded favourably to simple excision or mere shelling out of macroscopic tumour. Acid studies, serum gastrin and the secretin test became normal in all cases in the immediate postoperative period and remained so in three of the five patients when tested at 57, 33 and 8 months. The tests became abnormal in two patients when tested 8 and 53 months after surgery, but both patients are currently well-controlled on ranitidine 150 mg b.d. 22 and 65 months respectively after surgery. These results suggest that in patients with extra-pancreatic gastrinoma who have lymph node metastases simple excision of all macroscopic tumours may offer the prospect of long-term control. The place of total gastrectomy in such patients is questioned.
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PMID:Favourable response to conservative surgery for extra-pancreatic gastrinoma with lymph node metastases. 356 10

The patient described here, with malignant non-beta islet cell tumor of the head of the pancreas, was treated by resection of the tumor and metastases. Additional pathology of perforated duodenal ulcer and pyloric stenosis required vagotomy and pyloroplasty. The maintenance of normal gastrin levels after the operation indicates a good prognosis. We believe that the low-risk Zollinger-Ellison patient should be treated surgically and the tumor removed. When no tumor can be detected, parietal cell vagotomy should be performed to assist the pharmacological control of the gastric acid hypersecretion. Extensive surgery, such as total gastrectomy, is no longer the treatment of choice and is reserved for the so-called "cimetidine failure."
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PMID:Conservative surgery in Zollinger-Ellison syndrome: report of a case with an eight-year follow-up. 359 93

Treatment with omeprazole was evaluated in nine patients with the Zollinger-Ellison syndrome, in whom the effect of H2-receptor antagonists had become inadequate. Treatment with 20-80 mg omeprazole daily reduced basal acid secretion by 77-100%. The effect persisted during a continuous treatment of up to 2 years. In five patients the initial dose could be reduced after some time of treatment. In eight patients the treatment promptly relieved all symptoms, and in the last patient, who had disseminated metastatic disease and large anastomotic ulcers, the symptoms disappeared gradually over a period of 10 weeks. No adverse events were seen. We conclude that omeprazole is an effective inhibitor of the acid hypersecretion in Zollinger-Ellison patients, also when H2-receptor antagonists have failed.
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PMID:Omeprazole in the Zollinger-Ellison syndrome. 371 2

Eight cases of the Zollinger-Ellison syndrome were diagnosed at St Vincent's Hospital in the period 1966-84. Although a rare tumour, its true incidence is almost certainly greater than the number of cases represented in this series. The Zollinger-Ellison syndrome should be suspected in all cases of recurrent peptic ulceration, in cases of peptic oesophagitis not responding to medical treatment, in some cases of diarrhoea and in those cases of peptic ulceration associated with hypercalcaemia. Rarely the gastrinoma may first present as a mass in the head of the pancreas causing obstructive jaundice. Diagnosis has been made easier by estimation of fasting serum gastrins and the use of the secretin test. Localization is difficult. The treatment of the condition remains contentious. In those cases shown to be harbouring a so-called solitary gastrinoma, laparotomy should be performed with a view to resection. If the gastrinoma cannot be localized then it is reasonable to use H2 blocking agents to control hypersecretion. The presence of hypercalcaemia due to hyperparathyroidism must be controlled by parathyroidectomy. Total gastrectomy is reserved for those few cases who for one reason or another are not controlled by adequate H2 blocking therapy. In the presence of malignant gastrinoma with metastatic disease, hypersecretion is controlled by the use of H2 blocking agents. In this group cytotoxic chemotherapy may be used in an attempt to control the mass effects of the tumour.
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PMID:The Zollinger-Ellison syndrome: a review of the St Vincent's Hospital, Melbourne experience. 386 9

From 1978 to 1984, six patients (four men and two women) with functioning extrapancreatic gastrinomas were treated at the University of Michigan Medical Center. In all but one patient, who was known to have a liver metastasis, selective portal and peripheral venous sampling was performed to localize the site(s) of increased gastrin levels. Serum levels of carboxy (C) and amino (N) terminal gastrin moieties were measured before surgery with region-specific antisera and N/C ratios were calculated. All patients underwent operative exploration and successful resection of all gastrinomas present including a single large liver metastasis in one case. The C-terminal gastrin level was elevated in all cases and the N/C ratios were well below 1.0 (greater than 1 is consistent with malignancy and metastases) in all but the one patient with a liver metastasis. All patients have remained clinically free of tumor and have had normal basal and secretin-stimulated gastrin levels for as long as 5 years after operation. Three of the patients had undergone previous total gastrectomies and had done well apart from persistent hypergastrinemia before resection of the tumor. Two patients have not undergone any type of gastric operation. Appropriately selected patients with Zollinger-Ellison syndrome, particularly those with extrapancreatic primary lesions, may be candidates for a curative resection.
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PMID:Extrapancreatic gastrinomas. 407 87

Insulinoma, glucagonoma, gastrinoma (Zollinger-Ellison syndrome), vipoma, somatostatinoma and a tumor that secretes human pancreatic polypeptide are the primary endocrine-secreting tumors of the pancreas. hormones are produced by specific tumor cell types and cause a variety of dramatic clinical pictures. Diagnosis often requires hormone assays. Computerized tomography may be helpful. Definitive surgical treatment is possible, but metastases may be present.
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PMID:Primary endocrine-secreting pancreatic tumors. 610 68

Review of a 5 year clinical experience with the histamine H2-receptor antagonists metiamide, cimetidine, and ranitidine in 20 patients with Zollinger-Ellison syndrome disclosed a treatment failure rate of 50 percent. The criterion for failure was hemorrhage in four patients, obstruction followed by hemorrhage in one patient, perforation in one, and intractable pain in four. Nine of the 10 patients in whom treatment failed required total gastrectomy for control of complications; the 10th patient refused operation. Retrospective analysis identified hepatic metastases, the multiple endocrine adenomatosis-type I syndrome, refractory diarrhea, and breaks in the medication schedule as being more common in the treatment failure group, but these trends were not statistically significant in our small series of patients. Nonhealing or recurrent ulcers were found in 90 percent of the patients in whom drug therapy failed and in only 10 percent of those patients in whom therapy was successful (p less than 0.01). There were no differences related to age, sex, duration of symptoms, previous gastric operation, ulcer location, presence of diarrhea, or amount of drug prescribed. Basal and peak acid outputs, basal serum gastrin levels, and response to secretin challenge were also nondiscriminatory. The degree of acid inhibition in response to cimetidine was highly variable from one patient to another and on repeat testing in individual patients, and there was no correlation between acid secretory inhibition and clinical course. When severe complications occurred, reinstituting H2-receptor antagonist therapy or increasing the dose did not avert the need for total gastrectomy. Patients refractory to drug treatment who have persistent or recurrent ulcers should be managed with prompt total gastrectomy to prevent life-threatening complications.
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PMID:Failure of histamine H2-receptor antagonist therapy in Zollinger-Ellison syndrome. 612 12

Nine patients with the Zollinger-Ellison syndrome seen at a single referral centre between 1976 and 1981 are presented to highlight changes in the recognition, diagnosis and management of the condition. Less well recognized manifestations such as diarrhoea and features of the multiple endocrine neoplasia (MEN) type I syndrome are described, and the simplification of the pre-operative diagnosis by the use of both the serum gastrin estimation and the secretin provocation test considered. The problem of tumour localization is discussed with special reference to the newer techniques such as ultrasound, endoscopic retrograde cholangiopancreatography (ERCP) and CAT scanning, and the value of arteriography confirmed. The striking advances in management during the past few years are stressed with special reference to the role of the H2-receptor blocking drugs. Despite their profound inhibitory effect on both acid secretion and symptoms, all patients with the exception of those with proven metastases or the MEN type I syndrome underwent laparotomy to exclude a resectable lesion. If no resectable lesion was found truncal vagotomy was performed to facilitate acid secretory control post-operatively and H2-receptor blocking drugs continued in a dose necessary to maintain basal acid secretion under 5 mmol/hr.
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PMID:Changing concepts in the presentation, diagnosis and management of the Zollinger-Ellison syndrome. 613 24


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