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)

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

Exploratory laparotomy and a search for gastrinomas was performed in 52 patients with the Zollinger-Ellison syndrome (ZES). Gastrinoma tissue was resected in 11 patients (21%), 6 (12%) of whom appear to have been cured. After surgery, serum gastrin levels in these six patients have remained normal from 10 months to 10 years. In the 46 other patients, tumor was unresectable because of metastases or multiple primary tumors (21 patients; 40%) or inability to find the tumor at laparotomy (21 patients; 40%). Multiple pancreatic islet cell adenomata were found in six of seven patients with multiple endocrine neoplasia (MEN), indicating that patients with this condition usually have diffuse involvement of the pancreas. The results of CT scans correlated with findings at laparotomy in 13 of 16 patients. The smallest tumor detected by CT scans was 1 cm in diameter. CT technology is more accurate in finding gastrinomas now than in the past and has a useful role in preoperative evaluation. The possibility of resection should be seriously considered in every patient with Zollinger-Ellison syndrome. Abdominal CT scans, transhepatic portal venous sampling, and laparotomy should be used to find the tumor and to determine whether it is resectable. Using presently available methods, it should be possible to cure about 25% of patients with gastrinomas who do not have MEN and over 70% of those without MEN who appear to have a solitary tumor. Total pancreatectomy may be necessary to cure some patients with MEN, but that operation is rarely justified. The morbidity and mortality of surgical attempts at curing this disease have become minimal; we have had no deaths or serious complications following such operations in over 10 yrs. Total gastrectomy and indefinite use of H2-receptor blocking agents are the therapeutic options for patients with unresectable gastrinomas. Because H2-receptor blocking agents fail to control acid secretion in many patients after several yrs of therapy, total gastrectomy is indicated in a large proportion of patients whose tumors cannot be resected. Total gastrectomy in patients with ZES is also safe using current techniques; our last death following this operation for ZES occurred 15 yrs ago.
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PMID:Resection of gastrinomas. 613 3

Four patients with Zollinger-Ellison Syndrome (ZES) are presented to highlight the difficulties in the recognition, diagnosis and management of this rare disease. The presentation of ZES is usually indistinguishable from ordinary peptic ulcer disease and in those patients with symptoms not related to peptic ulcer, i.e., diarrhoea, as their main complaint, the diagnosis is often not even considered. A high index of suspicion is required, however, in patients with recurrent ulcers, multiple ulcers and in those with resistant or rapidly relapsing ulcers after conventional therapy. A presumptive diagnosis can be made by the demonstration of grossly elevated fasting serum gastrin levels combined with a secretin stimulation test in doubtful cases. The main problem is the location of the gastrin-secreting tumour which is usually pancreatic but often too small to be detected by currently available techniques. Histamine H2-receptor antagonists in high doses are effective in controlling the gastric acid hypersecretion which is chiefly responsible for the morbidity and mortality in ZES. They provide the treatment of choice in patients where the tumour cannot be located, though every attempt should be made to do this as surgery is the treatment of choice for this invariably malignant tumour. Total gastrectomy is now reserved for those patients in whom medical therapy has failed. The role of chemotherapy in metastatic disease has yet to be established.
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PMID:Zollinger-Ellison syndrome--report of four cases and review of literature. 614 89

The intestinal carcinoid tumors of 26 patients were stained for the presence of serotonin, gastrin, somatostatin, motilin, secretin, glucagon, pancreatic polypeptide, ACTH, and neurotensin. Argentaffin and argyrophil stains were also performed in all cases. Thirty-five separate tumors (counting metastases and multiple primaries) from the 26 patients were studied. Serotonin was present in 30 of the 35 tumors. Nineteen tumors contained serotonin only. Fourteen tumors contained multiple neuroendocrine products. One tumor contained gastrin only. One tumor did not stain immunohistochemically, but was argyrophilic. Metastatic deposits were studied in nine patients. Some metastases produced the identical neuroendocrine products as the primary tumor, whereas others produced either additional or fewer hormones than the primary tumor. Moreover, different metastases from the same primary tumor were observed to produce different hormones. Argyrophilic cells were present in all cases and were much more numerous than cells staining by immunohistochemistry. Argyrophilic cells probably contain monoamines and polypeptide hormones in addition to those studied in this series. The argyrophil stain was the best general stain in this study for the demonstration of neuroendocrine cells. Argentaffin staining was negative in ten cases that were serotonin positive and two argentaffin positive cases were serotonin negative. The carcinoid syndrome, as clinically defined by the presence of flushing and diarrhea, was noted in five patients, all of whom had serotonin-containing small bowel carcinoids. Endocrine-related symptoms were not clinically appreciated in the remaining patients.
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PMID:The neuroendocrine products of intestinal carcinoids. An immunoperoxidase study of 35 carcinoid tumors stained for serotonin and eight polypeptide hormones. 618 28

Records of the 30 cases of gastric carcinoid at the Mayo Clinic showed that the gastric mucosa was normal, hyperplastic, or atrophic (nonantral) in 12, 2, or 16 patients, respectively. In the atrophic group, the tumors were in the gastric body and fundus; small, polypoid, and multicentric; and associated with fundal argyrophil cell hyperplasia. In immunocytochemical studies, minor tumor cell populations stained positively for 5-hydroxytryptamine, gastrin, and somatostatin in 1 case and for 5-hydroxytryptamine in 3 others. Metastasis occurred in 3 patients. Twelve patients had pernicious anemia. Parietal cell or intrinsic factor antibodies or both were present in all 12 patients tested. Each of the 7 patients with an intact antrum had massive hypergastrinemia. No common HLA-A, -B, or -DR antigen pattern was detected among the 10 patients tested. The results suggest that nonantral gastric atrophy predisposes to gastric carcinoid as well as to gastric carcinoma.
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PMID:The syndrome of gastric argyrophil carcinoid tumors and nonantral gastric atrophy. 619 1

The main clinical signs of three dogs with Zollinger-Ellison syndrome were vomiting, diarrhoea, poor appetite and weight loss. The diagnosis was confirmed by histological examination and by gastrin immunocytochemistry. Gastrin was extracted from pancreatic tumours of two dogs. Gastrin-component III predominated in one dog while gastrin-component II and gastrin-component III were demonstrated in almost equal amounts in the other dog. In one dog serum gastrin concentration was high. Postmortem examination revealed pancreatic tumours in all three dogs and metastases in the regional lymph nodes and liver in two. The pancreatic tumours contained three patterns of growth: solid, trabecular and acinar. Electron microscopy of liver metastases showed cells with secretory granules. In all three dogs there was an erosive oesophagitis and thick gastric mucosa caused mainly by glandular proliferation. Two dogs had erosions and ulcers in the duodenum, one also in the first part of the jejunum. Villous atrophy and cellular infiltration of the duodenal mucosa were found in all dogs.
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PMID:Zollinger-Ellison syndrome in three dogs. 624 88

The authors report a case of glucagonoma in a 52 years old man presenting a migratory necrolytic erythema. By conjugated means of arteriography and splenoportography with plasma glucagon assays the tumour was localized in the tail of the pancreas. Surgical excision was easy but hepatic metastases revealed the malignant nature of the tumor. This glucagonoma has been investigated by several approaches including electron microscopy, immunocytochemistry and radioimmunological techniques. The tumor contained scattered glucagon and pancreatic polypeptide immunoreactive cells; insuline, glucagon, somatostatin, pancreatic polypeptide, gastrin and VIP antisera gave negative results. Ultrastructurally, these cells showed atypical secretory granules different from A granules of the normal glucagon cell. Radio immunological determinations carried out after gel permeation chromatography of plasma revealed high molecular weight (4 000, 9 000, 14 000) immunoreactive glucagon peptides. They have been thought to be proglucagon forms which did not react with specific antiglucagon sera used in cytological studies. Reported data are consistent with the classification of this tumor in the category of glucagonoma with the "glucagonoma syndrome".
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PMID:[A human pancreatic glucagonoma, ultrastructural, immunocytochemical and radioimmunological investigations (author's transl)]. 627 65

Decapeptide ceruletide (CRL), chemically related to cholecystokinin and gastrin, proved to have remarkable analgesic properties when administered to a group of 22 burned patients, 15 patients with acute myocardial infarction, and 8 patients suffering from pain caused by malignant tumours with metastases. Its effect was such, that many of the patients required no other analgesics (opiates) even after a prolonged administration (up to 10 days) of CRL. In some of the patients a marked euphoria developed. There were no substantial changes in EEG records during CRL administration in 15 controls, among them 4 epileptics. It is probable that CRL helps to activate the internal analgesic system. In the burned patients cortisol, testosterone, renin, prolactin and tri-iodothyronine (T3) levels in serum (plasma) were measured (radio-immunoassays). CRL did not block the stress response (no drop of increased cortisol levels, no increase in low T3 levels), but it modified (influenced) it (drop of the high renin levels, and a tendency to increase the very low testosterone levels). CRL appears to act as an endorphin releaser, as evidenced by the plasma levels of beta-endorphins (quotations). CRL and similar drugs may represent a new, more physiological and probably safer approach to the management of pain.
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PMID:Endorphin releasers: a new possible approach to the treatment of pain after burns--a preliminary report. 631 91

Peripancreatic venous abnormalities were demonstrated angiographically in 10 patients with islet cell tumor: six nonfunctioning, two gastrin-producing, one glucagon-producing, and one pancreatic polypeptide-producing. Venous involvement recognized included venous occlusion, venous encasement, and intraportal tumor growth. Seven patients had islet cell carcinoma with hepatic metastases while the other three had benign tumors. Three patients had arteriographic evidence of intraportal tumor growth with the "thread and streaks" sign, similar to that of portal venous extension of hepatocellular carcinoma.
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PMID:Venous involvement in islet cell tumors of the pancreas. 632 Jun 13

Twenty-five strictly defined bronchopulmonary carcinoids were studied by light microscopic immunohistochemistry by the peroxidase technique for NSE (neuron-specific enolase), serotonin, and a broad spectrum of neuropeptides. Eighteen cases were also studied by electron microscopy. Twenty-three of the twenty-five cases showed immunostaining for NSE; 24 cases displayed immunostaining for at least two of the hormones tested for; the single case that failed to show hormonal immuno-reactivity was however positive for NSE and had granules by electron microscopy. Serotonin was the most frequently demonstrated hormone followed by bombesin, vasoactive intestinal peptide, gastrin, leu-enkephalin , alpha-melanocyte stimulating hormone, somatostatin, substance P, and calcitonin. In several cases, adjacent-step sections stained for different hormones strongly indicated immunoreactivity for more than one hormone in single neoplastic cells. By electron microscopy, all 18 cases studied showed generally abundant neurosecretory granules, which, however, displayed considerable heterogeneity in their size, shape, and density. Twelve of these eighteen cases displayed evidence of squamous differentiation and 10 showed characteristic exocrine lumina. The capability of single neuroendocrine tumors and single neuroendocrine tumor cells to produce more than one immunoreactive hormone is hereby amply confirmed; these broad capabilities are certainly reflected in the heterogeneous granule populations seen by electron microscopy. The synchronous presence of squamous and exocrine features in broncho-pulmonary carcinoids indicates that they too are capable of multidirectional differentiation, which should not detract from their being regarded basically as well-differentiated neuroendocrine neoplasms. The clinical significance of strictly defining bronchial carcinoids is underscored by the fact that of 25 cases followed for 2-13 years, only one developed metastases 9 years postoperatively.
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PMID:Immunohistochemical and ultrastructural analysis of bronchopulmonary neuroendocrine neoplasms. I. Carcinoids. 637 57


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