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)

Recently a number of surgeons have recommended radical resection of gastrinomas in Zollinger-Ellison syndrome (ZES). We have developed a useful technique for preoperative localization of gastrinomas--the selective arterial secretin injection test (SASI)--and we recommend an intraoperative secretin test (IOS) for deciding the radicality of resection of gastrinomas. Here the results of SASI and IOS tests in 11 patients with ZES are examined and compared with the results of other techniques. The SASI test localized gastrinomas in all of the patients, while the sensitivity of ultrasonography, computed tomography, arteriography, or portal venous blood samplings was between 1/11 and 5/11. On the basis of the results of the SASI test, radical resection of gastrinoma was performed in four patients (three pancreatoduodenectomies and one extirpation). After pancreatoduodenectomy, immunohistologic study of the specimen revealed multiple microgastrinomas and lymph node metastases in two patients and the coexistence of a microgastrinoma and a gastinoma in one patient. The IOS test was useful in the estimation of the advisability of radicality, and in two patients total gastrectomy was not performed because of the results of the IOS test. These four patients are well and have returned to work, and their serum gastrin levels are below 35 pg/mL. Thus we believe SASI and IOS tests are helpful for planning curative resection of gastrinomas.
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PMID:Curative resection of multiple gastrinomas aided by selective arterial secretin injection test and intraoperative secretin test. 258 84

The treatment of patients with Zollinger-Ellison syndrome (ZES) has undergone dramatic evolution during the past decade. Although initially regarded as an incurable tumor, resection of gastrinoma for potential cure has been reported in 30% to 40% of selected patients in recent series. Conversely, although definitive control of acid hypersecretion is achieved by total gastrectomy, histamine (H2)-receptor antagonists and the newly introduced agents omeprazole and somatostatin analogues allow effective medical therapy of gastric acid overproduction. Confirmation of the diagnosis is best achieved with the I.V. secretin stimulation test, and tumor localization techniques are mandatory to identify candidates for operative tumor resection. Intraoperative sonography and careful exploration are required for tumor removal; successful tumor resection is associated with prolonged survival. The majority of patients (60%) are still found to have malignant disease at the time of diagnosis, but 10-year overall survival commonly exceeds 40%. The presence of multiple endocrine neoplasia type I (MEN-I) is seen in 10% to 25% of patients; correction of hypercalcemia alone may have therapeutic benefit in some ZES patients, and while gastrinoma resection is rarely possible, MEN-I patients demonstrate prolonged survival. The choice of medical rather than surgical therapy for acid hypersecretion depends on the suitability of each patient for careful and repeated endoscopic and chemical studies, versus the likelihood of a successful postoperative outcome. Socioeconomic, geographic, and related medical factors in each case may dictate the form of long-term antisecretory therapy. Exploration for possible tumor resection is indicated for virtually all patients who have no documented metastatic disease.
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PMID:Current diagnosis and management of Zollinger-Ellison syndrome. 268 66

A clinical and anatomic analysis was made of duodenal carcinoid tumors in 5 male patients (mean age 43.2 years). The tumors were present in the areas of the major duodenal papilla (in 3 cases), those of the bulb (1) and horizontal part of the duodenum (1). They were multiple in 2 cases. Clinically, signs of gastrin activity, i. e. recurring ulcers in various segments of the gastrointestinal tract (Zollinger-Ellison syndrome), were most common. Other changes included impairments in the biliary tract and liver. The causes of death were hepatic and renal insufficiencies (2 cases), thrombohemorrhagic events (2). In the closed glands, nesidioblastosis and thyroid C cell hyperplasia were detected, one case having type I multiple endocrinal neoplasia (adrenal corticosteroma, pancreatic apudoma). In three cases, the metastases invaded into the liver and lymph nodes. Direct correlation was not established between the tumor sizes, metastases and hormonal activity signs. All the tumors had not been recognized in the patients' life. The paper discusses if it is possible to make life-time diagnosis.
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PMID:[Clinico-anatomical comparisons in duodenal carcinoid tumors]. 274 30

The CT and magnetic resonance (MR) findings in 13 patients with advanced Zollinger-Ellison syndrome are described. In eight patients (62%) one or more primary tumors were found with both methods. All patients with proven liver metastases (n = 7) were identified by MR. Computed tomography was positive in six of these patients. Three patients with lymph node metastases were identified on CT and MR and one patient had bone metastases. Computed tomography and MR were inferior to selective arteriography in the detection of multiple lesions of the pancreas in a patient with multiple endocrine neoplasia-I syndrome. On the T1-weighted MR images, the primary tumors demonstrated no consistency with regard to their signal intensity relative to the adjacent pancreatic parenchyma. All gastrinomas had an increased relative signal intensity on the T2-weighted images with the exception of a calcified tumor. Liver and lymph node metastases had a low signal intensity on the T1-weighted images and an increased signal intensity on the T2-weighted images. The signal intensity of primary tumors and metastases was independent of size. In conclusion, MR was able to detect abnormalities based on its outstanding lesion/normal tissue contrast, whereas CT diagnosis was based mostly on contour distortion. For the current technique, MR is considered at least equal to CT.
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PMID:CT and MR imaging of advanced Zollinger-Ellison syndrome. 277 40

The potential for malignancy of an islet cell tumor of the pancreas is difficult to cytologically judge when one evaluates only the primary lesion, because a malignant condition is usually determined by the presence of regional or distant metastases. Nuclear DNA cytometric measurements have proved helpful both in the evaluation of the malignant potential of other endocrine and nonendocrine lesions and in the determination of the "aggressiveness" of these tumors. Thirty-six islet cell tumors or their metastases from 25 patients were studied. Eleven patients had insulinomas and typical insulinoma syndromes, and 14 others had gastrinomas with the Zollinger-Ellison syndrome. Tissue from each tumor was stained by the Feulgen technique, and nuclear DNA cytometry was performed by means of the microTICAS system designed by the Cytopathology Laboratory of the University of Chicago. Ploidy measurements of insulinomas, taken alone, did not discriminate well between benign and malignant states. However, the single malignant insulinoma could be clearly recognized, for it was one of only two lesions in that group with 5N-exceeding rate (5N-ER) values of 1% or greater. (5N-ER is defined as the percentage of aneuploid nuclei with nuclear DNA content greater than 5N.) On the other hand, seven of eight malignant gastrinomas had ploidy values of 2.5N or greater (our definition of an aneuploid state) and/or had 5N-ER values of 1% or greater, while five of six benign gastrinomas had ploidy values of less than 2.5N and had 5N-ER values of 0%. In addition, the two most aggressive tumors had the highest ploidy and 5N-ER values. Nuclear DNA cytometric studies appear to offer promise as an aid in the evaluation of pancreatic islet cell tumors, particularly gastrinomas.
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PMID:Nuclear DNA analysis of insulinomas and gastrinomas. 282 71

Forty-six patients with the gastrinoma syndrome were divided into 2 categories: 1) benign sporadic gastrinoma (n = 30), and 2) gastrinoma with metastases to liver (n = 16). Thirteen of the 46 patients had multiple endocrine neoplasia type I syndrome. Serum gastrin levels in patients fasted overnight were determined by RIA using antisera directed toward the NH2- and COOH-terminals of heptadecapeptide gastrin (G17) and the NH2-terminus of the triacontatetrapeptide (G34). These results were compared with findings in 50 normal subjects. In the normal subjects, the mean COOH-terminal gastrin-17 level was higher [65 +/- 8 (+/- SEM) pg/ml] than the NH2-terminal gastrin-17 level (11 +/- 0.2 pg/ml) and lower than the NH2-terminal gastrin-34 level (134 +/- 20 pg/ml). The levels of NH2-terminal gastrin-17 were higher in patients with metastatic disease than in those with benign gastrinoma, whereas the COOH-terminal gastrin-17 and the NH2-terminal gastrin-34 levels were similarly high in both groups. The mean ratio of NH2-terminal gastrin-17 to COOH-terminal gastrin-17 was less than 1 in normal subjects (0.22 +/- 0.02) and benign gastrinoma patients (0.2 +/- 0.04), and it was 2.2 +/- 0.41 in the patients with metastatic gastrinoma. An NH2 to COOH gastrin-17 ratio greater than 1 was found in 13 of 16 patients with metastatic gastrinoma, but in none of the patients with benign gastrinoma or normal subjects. Similar results were found in multiple endocrine neoplasia type I patients with benign and metastatic disease. A high NH2 to COOH gastrin-17 ratio is suggestive of metastatic gastrinoma. In 4 patients with metastatic gastrinoma, the NH2 to COOH gastrin-17 ratio fell in parallel with the response to chemotherapy.
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PMID:Evaluation of NH2-terminus gastrins in gastrinoma syndrome. 287 76

Patients with Zollinger-Ellison syndrome require that management decisions be made to control the gastric acid hypersecretion and treatment directed at the gastrinoma itself. The advent of newer antisecretory drugs and increased knowledge of the natural history of this disease have led to major changes in the management of each of these two areas. Recent studies have demonstrated that treatment with the currently available histamine H2-receptor antagonists (cimetidine, ranitidine) with or without an anticholinergic agent will control gastric acid secretion in almost all patients. These studies have also shown that most patients require higher doses than those used routinely to treat peptic ulcer, treatment is only successful if an adequate dose of antisecretory drug is used and must be monitored by measuring gastric acid hypersecretion, and established criteria to regulate the dose must be used. Newer more potent antisecretory drugs such as famotidine or omeprazole will facilitate management of gastric hypersecretion but are not yet currently available. Highly selective vagotomy should be considered in those patients who require high doses of cimetidine or ranitidine. Total gastrectomy should be reserved for those patients unwilling or unable to take oral medication. Although aggressive surgery is not warranted in most patients because overall prognosis is excellent, tumour status should be assessed in all patients by imaging studies (CT scan, ultrasound, selective angiogram). Patients without metastatic disease and without the MEN-1 syndrome (multiple-endocrine-neoplasia type 1) should undergo exploratory laparotomy by a surgeon experienced in treating this disease, with studies suggesting a cure rate of approximately 20%.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Current management of Zollinger-Ellison syndrome. 287 77

A center in Belfast, Northern Ireland, has established a register for tumors of the gastroenteropancreatic endocrine system. Carcinoid tumors occur most frequently. Of the non-carcinoid tumors, insulinomas, gastrinomas, and unknown types have the highest incidence, with other types being extremely rare. The potentially remediable nature of the tumors is stressed, and frequently a good quality of life can be experienced even in the presence of metastatic disease. The syndromes are probably underdiagnosed as they present with clinical features for which there are more common explanations, and appropriate diagnostic methods are therefore not used. The management of the syndromes is reviewed with particular emphasis on the treatment of patients with inoperable disease. Histamine (H2)-receptor antagonist therapy has made an impact in Zollinger-Ellison syndrome, and streptozotocin and somatostatin analogues can control tumor growth and endocrine syndromes, respectively.
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PMID:Neuroendocrine tumors. A European view. 287 46

Zollinger-Ellison syndrome is being detected at an earlier stage through liberal use of serum gastrin testing and application of secretin provocative tests if needed. The peptic ulcer disease of patients with Zollinger-Ellison syndrome can usually be controlled by large doses of one of the new potent gastric acid inhibitors. A battery of preoperative localizing tests can then be applied to guide exploratory laparotomy in non-MEN I patients. The tumor should be resected if possible, and continued low gastrin levels after operation provide evidence of a complete resection. It is reasonable to perform a parietal cell vagotomy at celiotomy because it will facilitate control of acid secretion if tumor resection is not successful. The only need for total gastrectomy is in a few patients whose acid secretion cannot be controlled with H2 receptor antagonists or who cannot comply with medical therapy. When no tumor is found at celiotomy, the prognosis for long-term tumor-free survival is excellent. Unfortunately, if unresectable hepatic metastases are present at operation, the patient is likely to die from metastatic tumor.
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PMID:Zollinger-Ellison syndrome (gastrinoma). Current diagnosis and treatment. 288 14

Medical treatment of the Zollinger-Ellison syndrome has been generally accepted because of the proven efficacy of the histamine (H2)-receptor antagonists in achieving symptomatic relief, and because of early reports indicating that few, if any, gastrinomas were resectable for cure. Gastrin radioimmunoassay (RIA) has made earlier and more certain diagnosis possible, and therefore reevaluation of the surgical management of gastrinomas is necessary. Experience with 60 gastrinoma patients is reported. Comparison between the pregastrin RIA years (before 1970) and post-gastrin RIA years was made to determine whether there was evidence to support the continuation of medical treatment without attempts to resect the gastrinoma. Twenty-five cases were diagnosed in the pre-RIA years. Age at diagnosis ranged from 17 to 68 years (median, 45 years). All patients were operated on. Metastases were found in 56 percent. No tumor was identified in 8 percent. Tumor was resected for "cure" (normal fasting gastrin levels for two years postoperatively) in one patient. Seventeen patients have died, and tumor was the cause of death in 70 percent. The five-year survival rate was 44 percent; the 10-year survival rate was 40 percent. Thirty-five cases were diagnosed after 1970. Age at diagnosis ranged from 39 to 61 years (median, 46 years). Thirty patients were operated on. Metastases were identified in 23 percent and no tumor was found in 17 percent. Tumor was resected for "cure" in 30 percent of patients. Seven patients have died and tumor caused death in 42 percent. The five-year survival rate was 82 percent; the 10-year rate was 64 percent. Advances in diagnosis and surgical technique since 1970 have made early operative treatment applicable in patients with gastrinoma. Because death in most cases is caused by progression of the tumor, an aggressive surgical approach to resect the tumor is advised soon after the diagnosis of Zollinger-Ellison syndrome is established.
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PMID:Early surgical treatment of gastrinoma. 288 76


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