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

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

The therapeutic principles in the management of endocrine gastroenteropancreatic (GEP) tumours include surgical extirpation of the primary tumour in the absence of metastases and medical control of symptoms in the preoperative phase. In the presence of metastases only palliative procedures are available. Tumour growth might be controlled by surgical procedures as debulking of tumour masses, medically by chemotherapy and more recently by new developments as a long-acting somatostatin analogue (SMS 201-995) and alpha-interferon. Their efficacy is currently evaluated in prospective studies. In contrast to inhibition of growth symptoms derived from excessive hormone production by GEP tumours can be well controlled. SMS 201-995 effectively prevents or at least improves flush and diarrhoea in the carcinoid syndrome, disabling diarrhoea in the Verner-Morrison syndrome and migratory erythema in the glucagonoma syndrome. SMS acts by inhibition of hormone release from the tumour and by a direct mechanism at the site of the target cell via SMS receptors present on tumour and target cells. For control of acid hypersecretion in gastrinoma patients omeprazole is superior to all former and present alternatives and replaced total gastrectomy completely. A similarly effective drug to prevent hypoglycaemia due to uncontrolled insulin release from insulinomas is not available since neither SMS nor diazoxide are effective in every insulinoma patient.
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PMID:Therapeutic strategies in the management of endocrine GEP tumours. 170 88

We reviewed the results of 187 consecutive ultrasound-guided fine-needle biopsies of the pancreas in 171 patients to assess the diagnostic accuracy of the method. The final diagnosis obtained at operation, autopsy or follow-up were: adenocarcinoma (n = 83), metastatic cancer (n = 11), cystadenocarcinoma (n = 2), lymphoma (n = 2), malignant gastrinoma (n = 1), pseudocyst (n = 25), cyst (n = 13), chronic pancreatitis (n = 9), normal pancreas (n = 10), abscess (n = 7), benign islet-cell tumour (n = 5), cystadenoma (n = 3). Sufficient cytologic material was obtained in 95.3% of biopsies and the overall accuracy in distinguishing benign from malignant disease was 85.4%. False negative results were obtained in 12 patients (13.1%). Inconclusive results (CIII) were found in aspirates from one cyst and two islet cell tumours. There were no false-positive results. The only complication was a post-biopsy haematoma around the head of pancreas, which resolved spontaneously. Ultrasound-guided pancreatic fine-needle biopsy is a safe method and allows of a high degree of diagnostic accuracy. It has a high specificity. Its sensitivity in the detection of malignancy improves if biopsies are repeated in doubtful cases. It further permits tumours to be graded and allows complications of pancreatitis to be diagnosed.
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PMID:Diagnostic accuracy of ultrasound-guided fine-needle pancreatic biopsy. 173 79

A 29-year old woman presented with neuro-lupus and hepatic metastases of gastrinoma. We suggest that this hitherto undescribed association might not be fortuitous.
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PMID:[Neuro-lupus associated with Zollinger-Ellison syndrome with hepatic metastases: an incidental association?]. 178 55

Because of its widespread distribution within the nervous system and the gastro-enteropancreatic (GEP) system and its diverse physiological inhibitory actions on various gastrointestinal functions, including endocrine and exocrine secretion, motility, liver and splanchnic blood flow and absorption, native somatostatin has been viewed as a possible therapy for many diseases. However, its short duration of action and consequent limited clinical usefulness have been overcome with the availability of Sandostatin, a long-acting, synthetic octapeptide analogue of the naturally occurring hormone. Sandostatin represents a significant advance in the treatment of GH and TSH secreting pituitary tumours and GEP endocrine tumours (carcinoid tumour, VIPoma, glucagonoma, insulinoma, and gastrinoma). Preclinical in vitro and animal studies have shown the antineoplastic activity of the compound. Moreover, because of a possible direct effect on somatostatin receptor-positive endocrine tumour cells and indirect effect whereby Sandostatin lowers GH, IGF-1 and numerous gastrointestinal peptides, Sandostatin may prove useful as an adjunctive therapy in cancer patients. In vivo labelling of somatostatin receptor-positive tumours with radiolabelled somatostatin analogues now allows localisation of such tumours and their metastases. Moreover, targeted irradiation of these tumours by beta particle emitting isotopes attached to such somatostatin analogues may become possible. The use of Sandostatin in acute oesophageal variceal bleeding, pancreatic pseudocysts, gastrointestinal and pancreatic external fistulae, short bowel syndrome, dumping syndrome and AIDS-related refractory hypersecretory diarrhea has provided encouraging results. Preliminary reports indicate efficacy of Sandostatin in psoriasis, autonomic neuropathy (postprandial and orthostatic hypotension) and its ability to reduce height velocity in tall adolescents. The ultimate role of Sandostatin as a therapeutic agent in these disorders is being explored in prospective clinical trials.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Future medical prospects for Sandostatin. 198 Jul 78

Surgical treatment of hepatic metastases is predominantly aimed at "curative" resection. This can be achieved in about 20% of colorectal secondaries, and is associated with a 30-40% 5-year survival. One to three metastases in the absence of extrahepatic disease are regarded a clear indication to resection. Among patients with non-colorectal malignancies, occasional long term survival was reported in leiomyosarcoma, breast cancer, and renal cancer metastases, respectively. Endocrine tumors such as carcinoid, gastrinoma, or pheochromocytoma, are different because of their remarkable symptoms along with a protracted natural history. Quality of life may be considerably improved here by even non-radical debulking. The vast majority of patients, however, ist not suitable to undergo hepatic resection. Palliative therapeutic options involve hepatic artery ligation or embolization, cryo-surgery and percutaneous laser coagulation, and various types of regional chemotherapy. These methods may enable a temporary relief of symptoms, but no significant impact on survival time, and no true long term benefit has been proven. Prospective randomized trials against combined treatment as well as untreated patients are required for a more meaningful judgement and improved effectiveness.
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PMID:[Surgical interventions in liver metastases]. 198 54

Percutaneous ethanol injection (PEI) was performed under ultrasound (US) guidance in the outpatient department in 14 patients with 21 metastases 1.0-3.8 cm in diameter. The primary cancer was colorectal adenocarcinoma (seven patients), adenocarcinoma of the stomach (four patients), abdominal leiomyosarcoma (one patient), gastrinoma of unknown origin (one patient), and bronchial carcinoid (one patient). The indicators of therapeutic efficacy were US, computed tomography, fine-needle biopsy, and serologic markers. No complications occurred after a total of 175 treatment sessions. Complete response was obtained in 11 lesions, nine of which were less than 2 cm in diameter, and in all endocrine metastases (four lesions in two patients) with a maximum, recurrence-free follow-up of 38 months. Carcinoembryonic antigen values decreased in all patients but one for a 2-6-month period. The natural course of metastatic disease strictly limits the applicability of PEI, a local treatment. Single, metachronous, nonoperable metastasis of adenocarcinoma and endocrine metastases seem to be the only indications for PEI.
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PMID:Liver metastases: results of percutaneous ethanol injection in 14 patients. 202 79

In six patients with hepatically metastasised gastrointestinal tumours of neuro-endocrinal origin, including one each islet cell carcinoma and gastrinoma of the pancreas, one cloacogenic carcinoma (a rare type of anal cancer), and three carcinoid tumours of the small bowel, spontaneous liquefaction inside echogenic liver metastases was demonstrated by ultrasound. The hepatic lesions were classified as being of "bull's-eye-" shape without "badge" pattern. Chemotherapy had no effect on their ultrasonographic imaging. With regard to our observations, liquefying processes were far more common in these well-differentiated tumour metastases than in poorly differentiated fast-proliferating malignancies (about 1:40). Assessment of liquefaction, however, has obviously no influence on the prognosis of progress speed. Ultrasonographic differential diagnosis includes focal inflammatory disease of the liver, if there is no evidence of a primary tumour.
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PMID:[Spontaneous regression of liver metastases in neuroendocrine tumors of the abdomen]. 205 55

As clinical experience with patients with ZES has grown, increasing recognition has been made of the broad spectrum of symptoms associated with gastrinomas. Diarrhea and acid-induced esophageal injury have taken their place alongside chronic peptic ulcer disease as indications for screening for gastrinoma. Diagnostic testing should begin with fasting serum gastrin levels and should include intravenous secretin infusion if fasting serum levels of gastrin are nondiagnostic and the patient is not found to be hypochlorhydric. Tumor localization is critical to aid in the identification of patients with potentially curable localized disease. Preoperative evaluation utilizing CT scanning with intravenous contrast should be done early and should be supplemented by other imaging modalities as necessary. Exploratory laparotomy, including a thorough examination of the duodenum and perhaps intraoperative ultrasound, should be performed in all patients with sporadic gastrinoma who lack evidence of extensive metastatic disease on preoperative evaluation. By utilizing this approach, it is likely that at least 20% of patients with ZES can be cured. With the availability of the highly effective H(+)-K(+)-ATPase inhibitor omeprazole, excellent control of symptoms related to gastric acid hypersecretion can be expected. Patients with unresectable gastrinoma may thus avoid potentially morbid antisecretory surgery and be managed with a fairly simple medical regimen. Further developments in the chemotherapeutic management of these patients with unresectable disease should be forthcoming in the future.
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PMID:Zollinger-Ellison syndrome. 207 95


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