vasoactive-intestinal-peptide and Zollinger-Ellison-Syndrome

The application of radioimmunoassay of insulin, C-peptide, gastrin, glucagon, vasoactive intestinal polypeptides (VIP), somatostatin, human pancreatic polypeptides (hPP), substance P and neurotensin to detect endocrine tumors of the pancreas and other organ systems is undoubtedly important in the clinical management of patients suspected of having tumors that secrete these hormones. Radioimmunoassays of the above gut peptides have certain degrees of specificity and sensitivity; however, there are several factors that need to be considered in the interpretation of results since heterogeneity of molecular forms does occur and the varied radioimmunoassay techniques use different antibodies that may yield different results. It is, therefore, important that each laboratory establish its own normal values, determine the molecular species that each assay is detecting, and also determine the false positivity of the methodology. The same endocrine tumor may contain and secrete several detectable peptides, but the syndrome may relate to only one peptide. Although the simultaneous measurement of multiple peptides in patients with benign gastrointestinal disease has yielded information that contributes to our understanding of the complexities of gut neuroendocrine interaction, the pathophysiological role of gut peptides and their clinical relevance need further evaluation.

Topics: Animals; Diagnosis, Differential; Digestion; Food; Gastrins; Gastrointestinal Diseases; Gastrointestinal Hormones; Humans; Kidney Diseases; Pancreatic Neoplasms; Pancreatic Polypeptide; Radioimmunoassay; Vagus Nerve; Vasoactive Intestinal Peptide; Vipoma; Zollinger-Ellison Syndrome

Gut peptide secreting tumours originate most commonly from the pancreatic Islets of Langerhans. Tumours at a variety of other sites have also been shown to synthesize and release these peptides, reflecting the wide distribution of the peptide secreting cells of the diffuse neuroendocrine system. Tumours such as the glucagonomas, insulinomas, VIPomas and gastrinomas are associated with characteristic clinical syndromes resulting from the effects of the peptide they secrete. The majority of the islet cell tumours in fact secrete a number of different peptides and many of these are present in several molecular forms, some of which may not be biologically active. This may explain the lack of clinical sequelae in association with tumours such as the somatostatinomas. The clinical features, methods of diagnosis, localisation and treatment of these tumours will be discussed.

Topics: Adenoma, Islet Cell; Bombesin; Bronchial Neoplasms; C-Peptide; Carcinoma, Small Cell; Diagnosis, Differential; Endocrine System Diseases; Erythema; Gastrointestinal Hormones; Glucagon; Glucagonoma; Humans; Insulin; Insulin Secretion; Insulinoma; Male; Neoplasms; Neurotensin; Pancreatic Hormones; Pancreatic Neoplasms; Pancreatic Polypeptide; Somatostatinoma; Vasoactive Intestinal Peptide; Zollinger-Ellison Syndrome

Topics: Adenoma, Islet Cell; Female; Gastrins; Humans; Insulin; Insulin Secretion; Male; Pancreatic Hormones; Pancreatic Neoplasms; Pancreatic Polypeptide; Radioimmunoassay; Vasoactive Intestinal Peptide; Zollinger-Ellison Syndrome

Topics: Achlorhydria; Adenoma, Islet Cell; Adult; Aged; Diagnosis, Differential; Diarrhea; Female; Gastric Juice; Humans; Hypokalemia; Male; Middle Aged; Pancreatic Neoplasms; Syndrome; Vasoactive Intestinal Peptide; Zollinger-Ellison Syndrome

Topics: Adenoma, Islet Cell; Chenodeoxycholic Acid; Cholecystokinin; Cholelithiasis; Dehydration; Diabetes Mellitus; Duodenal Ulcer; Gastric Inhibitory Polypeptide; Gastrins; Gastrointestinal Hormones; Humans; Motilin; Pancreatic Neoplasms; Secretin; Syndrome; Vasoactive Intestinal Peptide; Zollinger-Ellison Syndrome

Topics: Bombesin; Cholecystokinin; Cimetidine; Epidermal Growth Factor; Gastric Inhibitory Polypeptide; Gastrins; Gastrointestinal Hormones; Humans; Motilin; Nerve Tissue; Pancreas; Pancreatic Polypeptide; Pituitary Gland; Secretin; Somatostatin; Stomach Neoplasms; Vasoactive Intestinal Peptide; Zollinger-Ellison Syndrome

The identification and description of a widely dispersed group of cells of common origin and biochemical characteristics, APUD cells, has allowed a better understanding and classification of endocrine tumors of the pancreas. Similarly, it has enabled the relationships between the endocrine tumors of the multiple endocrine neoplasia type I syndrome and the endocrine tumors of the pancreas to be better appreciated. This has facilitated both diagnosis and management of these conditions. The pluripotentiality of the cells of the APUD system combined with the certain existence of many unidentified peptides suggests the likelihood of other undescribed pancreatic endocrine tumors. Many of these are probably part of the heterogenous group of neoplasms currently designated as carcinoids, since their secretory products and exact cell types are not known. The recognition of the physiologic characteristics and cells of origin of these peptides, amines or other bioactive agents will allow delineation of the symptom complex and the identification of further functional tumors of the pancreas. The development of plasma radioimmunoassays for the various hormones and the appreciation of the specific clinical syndromes related to each tumor have enabled earlier diagnosis. The understanding of the hormonal physiopathologic functions has led to the evolution of specific therapeutic maneuvers. Provocative tests have allowed increased precision of the differential diagnosis, while selective arteriography and pancreatic venous sampling have greatly enhanced the accuracy of topical localization. The role of operation in tumor removal is still prominent, but malignant and recurrent tumors may now also be controlled with specific pharmacotherapy or appropriate endocrine cytotoxic agents. The use of peptides with antagonistic actions or the administration of specific antibodies to the active tumor products are areas of therapy that require further exploration.

Topics: Adult; Apudoma; Carcinoid Tumor; Child; Diagnosis, Differential; Gastrins; Glucagon; Humans; Infant; Insulin; Insulin Secretion; Islets of Langerhans; Neoplasms, Multiple Primary; Pancreatic Neoplasms; Pancreatic Polypeptide; Somatostatin; Vasoactive Intestinal Peptide; Zollinger-Ellison Syndrome

Topics: Adenoma, Islet Cell; Cholecystokinin; Gastric Juice; Gastrins; Gastrointestinal Hormones; Glucagon; Humans; Neoplasms; Pancreatic Neoplasms; Paraneoplastic Endocrine Syndromes; Peptides; Precancerous Conditions; Secretin; Somatostatin; Stomach Neoplasms; Vasoactive Intestinal Peptide; Zollinger-Ellison Syndrome

Topics: Acute Kidney Injury; Adenoma, Islet Cell; Adolescent; Adult; Aged; Carcinoid Tumor; Child; Dehydration; Female; Gastric Inhibitory Polypeptide; Gastrins; Gastrointestinal Hormones; Gastrointestinal Neoplasms; Glucagon; Humans; Hyperplasia; Hypokalemia; Insulin; Insulin Secretion; Male; Middle Aged; Pancreatic Neoplasms; Paraneoplastic Endocrine Syndromes; Precancerous Conditions; Serotonin; Somatostatin; Syndrome; Vasoactive Intestinal Peptide; Zollinger-Ellison Syndrome

Topics: Animals; Cholecystokinin; Dogs; Gastric Inhibitory Polypeptide; Gastric Juice; Gastrins; Gastrointestinal Hormones; Humans; Pancreatic Diseases; Radioimmunoassay; Secretin; Syndrome; Vasoactive Intestinal Peptide; Zollinger-Ellison Syndrome

Topics: Animals; Cholecystokinin; Dogs; Feedback; Gastric Inhibitory Polypeptide; Gastrins; Gastrointestinal Hormones; Humans; Secretin; Swine; Vasoactive Intestinal Peptide; Zollinger-Ellison Syndrome

Topics: Digestion; Esophageal Achalasia; Esophagitis, Peptic; Gastric Inhibitory Polypeptide; Gastric Mucosa; Gastrins; Gastritis; Glucagon; Humans; Ileum; Islets of Langerhans; Jejunum; Kidney Failure, Chronic; Liver; Protein Hydrolysates; Pyloric Antrum; Somatostatin; Vagotomy; Vasoactive Intestinal Peptide; Zollinger-Ellison Syndrome

Topics: Adenoma, Islet Cell; Carcinoid Tumor; Cholecystokinin; Diarrhea; Gastrins; Gastrointestinal Hormones; Gastrointestinal Neoplasms; Glucagon; Hormones, Ectopic; Humans; Insulin; Insulin Secretion; Neurosecretory Systems; Pancreatic Hormones; Paraneoplastic Endocrine Syndromes; Prostaglandins; Serotonin; Syndrome; Vasoactive Intestinal Peptide; Werner Syndrome; Zollinger-Ellison Syndrome

Over a five-year period, we measured concentrations of gut hormones in plasma samples from 353 patients in whom diagnoses of pancreatic endocrine tumors were subsequently confirmed. A median of 19 months (range, 7 to 120) after the initial diagnosis, 24 of these patients (6.8 percent) had elevated concentrations of other hormones in association with new clinical symptoms. In 13 of these patients (8 with glucagonomas, 3 with tumors secreting vasoactive intestinal polypeptide, and 2 with insulinomas), hypergastrinemia developed along with the clinical features of a gastrinoma; 5 patients died of gastrointestinal perforation or bleeding, apparently caused by this second tumor. We conclude that patients with pancreatic endocrine tumors, regardless of their initial clinical picture, require continued surveillance for new elevations of hormones.

Topics: Adenoma, Islet Cell; Adult; Aged; Gastrins; Gastrointestinal Hemorrhage; Glucagon; Glucagonoma; Hormones; Humans; Insulinoma; Middle Aged; Pancreatic Neoplasms; Time Factors; Vasoactive Intestinal Peptide; Vipoma; Zollinger-Ellison Syndrome

Long-acting somatostatin analogues such as SMS 201-995 (Sandoz) are being evaluated in a wide range of clinical indications, including gut neuroendocrine tumours and acrogemaly. Long-term continuous SMS 201-995 treatment has achieved useful symptomatic improvement in diarrhoea in 4 patients with metastatic VIPomas who had relapsed following previous treatment. Clinical improvement has outlasted suppression of VIP secretion (suggesting an additional direct antisecretory action of SMS 201-995) and has occurred despite expansion of hepatic metastases. In 6 patients with tumours secreting gastrin and/or glucagon, secretion of these peptides was acutely inhibited by SMS 201-995. However, endocrine and clinical responses to chronic treatment have been less consistent. SMS 201-995 is active orally at doses of 4-8 mg and when given thrice-daily to 6 patients with active acromegaly, suppressed mean 24-h growth hormone levels by 51-88%. Despite significantly reduced plasma insulin concentrations, glucose tolerance did not deteriorate. SMS 201-995 was also effective in suppressing thyroid-stimulating hormone (TSH) and thyroid hormone secretion in a patient with mild thyrotoxicosis due to non-tumoural inappropriate TSH hypersecretion. In all cases SMS 201-995 treatment has been well tolerated and has few side-effects.

Topics: Acromegaly; Adenoma; Adult; Aged; Diarrhea; Female; Gastrointestinal Neoplasms; Glucagonoma; Growth Hormone; Humans; Hyperthyroidism; Liver Neoplasms; Male; Middle Aged; Neoplasms, Glandular and Epithelial; Octreotide; Pancreatic Neoplasms; Pituitary Neoplasms; Somatostatin; Streptozocin; Thyrotropin; Vasoactive Intestinal Peptide; Vipoma; Zollinger-Ellison Syndrome

Topics: Diagnosis, Differential; Humans; Hyperparathyroidism; Insulinoma; Multiple Endocrine Neoplasia; Pancreatic Neoplasms; Prolactin; Vasoactive Intestinal Peptide; Zollinger-Ellison Syndrome

Topics: Cholecystokinin; Gastrins; Gastrointestinal Diseases; Gastrointestinal Hormones; Humans; Vasoactive Intestinal Peptide; Zollinger-Ellison Syndrome

The case history of a patient with an islet cell carcinoma, which produced both gastrin and vasoactive intestinal polypeptide (VIP), is presented. Although several examples have been observed of the combined production of these hormones by pancreatic endocrine tumors, few reports have related the clinical details of such cases. Resolution of diarrhea occurred in our patient after institution of nasogastric suction and cimetidine therapy, suggesting that gastric hypersecretion, rather than VIP activity, accounted for this problem. Chemotherapy with streptozotocin and 5-fluorouracil was highly effective in ameliorating clinical symptoms, diminishing serum levels of gastrin and VIP, and greatly reducing the bulk of metastatic disease in this case.

Topics: Adenoma, Islet Cell; Antineoplastic Combined Chemotherapy Protocols; Female; Fluorouracil; Gastrins; Humans; Middle Aged; Pancreatic Neoplasms; Streptozocin; Vasoactive Intestinal Peptide; Zollinger-Ellison Syndrome

Topics: Adenoma, Islet Cell; Adult; Female; Gastrins; Glucagon; Humans; Hyperplasia; Islets of Langerhans; Male; Middle Aged; Multiple Endocrine Neoplasia; Pancreatic Neoplasms; Pancreatic Polypeptide; Phosphopyruvate Hydratase; Serotonin; Somatostatin; Staining and Labeling; Vasoactive Intestinal Peptide; Zollinger-Ellison Syndrome

Topics: Endocrine System Diseases; Enterochromaffin Cells; Female; Gastrointestinal Neoplasms; Glucagonoma; Humans; Insulinoma; Male; Multiple Endocrine Neoplasia; Pancreatic Neoplasms; Somatostatinoma; Stomach Neoplasms; Vasoactive Intestinal Peptide; Zollinger-Ellison Syndrome

Topics: Adenoma, Islet Cell; Humans; Insulinoma; Pancreatic Neoplasms; Vasoactive Intestinal Peptide; Vipoma; Zollinger-Ellison Syndrome

Topics: Adrenal Glands; Adult; C-Peptide; Carcinoid Tumor; Epidermolysis Bullosa; Female; Gastrointestinal Diseases; Gastrointestinal Hormones; Gastrointestinal Neoplasms; Glucagonoma; Humans; Male; Middle Aged; Multiple Endocrine Neoplasia; Somatostatinoma; Thyroid Gland; Vasoactive Intestinal Peptide; Vipoma; Zollinger-Ellison Syndrome

In three families with the multiple endocrine adenomatosis type I (MEA I) trait, 51 members were investigated by measurement of circulating peptide hormones as tumor markers. Twenty-five of 51 members (49 percent) were considered to be affected by MEA I disorders. The incidence rose with age (75 percent in generation II). Both sexes were affected equally. Hyperparathyroidism was present in 20 of 25 affected members (80 percent), and pituitary tumors (prolactinomas) were found in four of 25 (16 percent). Endocrine pancreatic tumors were found in nine of 25 affected members (36 percent), but when "probable" tumors (seven) are included the frequency rises to 72 percent. Hyperparathyroidism was found in all except one member with proved lesions in other organs. Among patients with proved and possible endocrine pancreatic tumors, elevated serum levels of gastrin and pancreatic polypeptide were frequently found, 78 percent and 67 percent, respectively, and we suggest that serum gastrin and pancreatic polypeptide levels are the most useful screening markers at present for pancreatic lesions in MEA I.

Topics: Adenoma; Adolescent; Adult; Age Factors; Aged; Female; Gastrins; Humans; Hyperparathyroidism; Insulinoma; Liver Neoplasms; Male; Middle Aged; Multiple Endocrine Neoplasia; Pancreatic Neoplasms; Pancreatic Polypeptide; Parathyroid Neoplasms; Pedigree; Vasoactive Intestinal Peptide; Zollinger-Ellison Syndrome

The mechanisms by which bacterial enterotoxins cause secretory diarrheas have been well defined, and the definitions of such mechanisms have been important in developing a consistently successful therapeutic approach. The less common secretory diarrheas, caused by the interaction of hormones of tumor origin with the gut small intestinal mucosa have also been clearly defined, and their pathogenetic mechanisms are similar to those by which the cholera and E. coli enterotoxins cause secretory diarrhea. The mechanisms by which histamine and gastrin of tumor origin cause gastric hypersecretion are less clearly delineated; secretory diarrhea caused by both of these agents can be stopped by total gastrectomy without removal of the responsible tumor. The secretory diarrhea caused by villous adenomas of the colon, which does not appear to be related to a distally produced humoral agent, results in the same picture of hypokalemic acidosis that is characteristic of the nonbacterial secretory diarrheas originating in the small intestine and is cured by resection of the responsible tumor.

Topics: Adenoma; Antigens, Bacterial; Bacterial Toxins; Cholera; Cholera Toxin; Colonic Neoplasms; Diarrhea; Enterotoxins; Escherichia coli; Escherichia coli Infections; Escherichia coli Proteins; Fimbriae Proteins; Hormones, Ectopic; Humans; Hydrogen-Ion Concentration; Intestinal Mucosa; Neoplasms, Hormone-Dependent; Prostaglandins; Vasoactive Intestinal Peptide; Water-Electrolyte Balance; Zollinger-Ellison Syndrome

Topics: Gastrointestinal Hormones; Glucagonoma; Humans; Insulinoma; Intestinal Mucosa; Somatostatinoma; Vasoactive Intestinal Peptide; Zollinger-Ellison Syndrome

One hundred twenty-five pancreatic endocrine tumors were analyzed by immunocytochemistry using various antisera. Twenty-three of 27 insulinomas, 10 of 10 PP-omas (PP: pancreatic polypeptide) and 15 of 30 "nonsecreting" tumors were benign, whereas 8 of 13 glucagonomas, 16 of 24 gastrinomas, and 16 of 21 VIP-omas (VIP: vasoactive intestinal polypeptide) were malignant. As a rule, the hormone secreted by the tumor and causing clinical symptoms could be localized by immunocytochemistry. Fifty of 95 active tumors were found to contain cells immunoreactive to peptide(s) not causing clinical symptoms, and 54 of 30 "nonsecreting" tumors were shown to be multicellular. By electron microscopy more than one cell type could be identified in 12 tumors. Histologically, the growth pattern of the tumors was very variable and distribution of immunoreactive cells was distinctly patchy. Radioimmunoassay on extracts of 20 of 27 tumors confirmed the presence of peptides visualized by immunocytochemistry. In 17 of 22 specimens, groups of endocrine cells in close contact with ductules were found in the pancreatic parenchyma distant from the tumor. Pancreatic endocrine tumors probably arise from the pancreatic ductular epithelium. They are often multicellular, producing and sometimes secreting more than one hormone or hormone-like substance. They represent highly complex biologic systems in which the interrelationship of various gastrointestinal-pancreatic hormones can be studied.

Topics: Adolescent; Adult; Aged; Child; Endocrine System Diseases; Female; Glucagonoma; Humans; Insulinoma; Male; Middle Aged; Pancreatic Neoplasms; Pancreatic Polypeptide; Vasoactive Intestinal Peptide; Zollinger-Ellison Syndrome

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.

Topics: Adenoma, Islet Cell; Apudoma; Gastrins; Glucagon; Humans; Insulin; Insulin Secretion; Pancreatic Neoplasms; Pancreatic Polypeptide; Somatostatin; Vasoactive Intestinal Peptide; Zollinger-Ellison Syndrome

Topics: Adenoma, Islet Cell; Carcinoid Tumor; Digestive System Neoplasms; Gastrointestinal Hormones; Hormones, Ectopic; Humans; Pancreatic Neoplasms; Somatostatin; Vasoactive Intestinal Peptide; Zollinger-Ellison Syndrome

Qualitative and quantitative immunocytochemistry, electronmicroscopy and radio-immuno-assays led to the discovery of 5 pancreatic polypeptide hormones under physiological conditions. The active endocrine cells and the produced hormones are termed A, B, D, D1, and PP cell and glucagon, insulin, somatostatin, vasoactive intestinal polypeptide (VIP) and pancreatic polypeptide (PP) respectively. Beside the physiology of secretion and action a survey of pathological conditions in the paediatric age group is given. Insulin is the most important of pancreatic hormones in childhood. Therefore diagnosis and treatment of hyperinsulinism are described in extension.

Topics: Child; Glucagon; Humans; Hyperinsulinism; Insulin; Pancreatic Hormones; Pancreatic Polypeptide; Vasoactive Intestinal Peptide; Zollinger-Ellison Syndrome

Topics: Adenoma, Islet Cell; Dehydration; Diagnosis, Differential; Diarrhea; Gastrointestinal Hormones; Humans; Hypokalemia; Male; Middle Aged; Pancreatic Neoplasms; Paraneoplastic Endocrine Syndromes; Vasoactive Intestinal Peptide; Zollinger-Ellison Syndrome

Topics: Amino Acid Sequence; Animals; Brain Chemistry; Cholecystokinin; Dogs; Gastric Acid; Gastric Juice; Gastrins; Gastrointestinal Hormones; Humans; Swine; Vasoactive Intestinal Peptide; Zollinger-Ellison Syndrome

Topics: Adenoma, Islet Cell; Adult; Female; Gastrins; Gastrointestinal Hormones; Glucagon-Like Peptides; Humans; Male; Middle Aged; Multiple Endocrine Neoplasia; Pancreatic Neoplasms; Somatostatin; Vasoactive Intestinal Peptide; Zollinger-Ellison Syndrome

Topics: Adenoma, Islet Cell; Carcinoid Tumor; Gastrointestinal Neoplasms; Glucagon; Growth Hormone-Releasing Hormone; Humans; Pancreatic Polypeptide; Peptide Biosynthesis; Vasoactive Intestinal Peptide; Zollinger-Ellison Syndrome

A father and son each presented with severe watery diarrhea. The son was found to have a pancreatic islet-cell tumor associated with the pancreatic cholera syndrome, as well as a parathyroid adenoma. The father was found to have multiple islet-cell adenomas and the Zollinger-Ellison syndrome. Pancreatic tumor tissue from each patient contained detectable gastrin and vasoactive intestinal peptide; however, a much higher gastrin concentration was found in the tumor tissue from the father and a much higher vasoactive intestinal peptide content in the tumor tissue from the son. Thus, watery diarrhea may be mediated by different hormones in families having multiple endocrine neoplasia; the precise cause of the diarrheal syndrome should be defined to ensure the proper therapy.

Topics: Adenoma; Adenoma, Islet Cell; Adult; Diarrhea; Endocrine System Diseases; Gastrins; Humans; Male; Middle Aged; Neoplasms, Multiple Primary; Pancreatic Neoplasms; Parathyroid Neoplasms; Vasoactive Intestinal Peptide; Zollinger-Ellison Syndrome

Pancreas and gut hormones are involved in many endocrine and gastrointestinal diseases. Radioimmunoassays for these hormones have proved particularly valuable in diagnosis, localisation and control of treatment of endocrine tumours, of which many are mixed. An estimate based on ten years experience in a homogenous population of 5 million inhabitants (Denmark) suggests, that endocrine gut tumour-syndromes on an average appear with an incidence of 1 patient per year/syndrome/million. At present six different syndromes are known: 1) The insulinoma syndrome, 2) The Zollinger-Ellison syndrome.3) The Verner-Morrison syndrome. 4) The glucagonoma syndrome. 5) The somatostatinoma syndrome, and 6) the carcinoid syndrome. Accordingly diagnostically valuable RIAs for pancreas and gut hormones include those for insulin, gastrin, VIP, HPP, glucagon, somatostatin, and presumably also substance P. It is probably safe to predict that the need for gut and pancreas hormone RIAs within the next decade will increase greatly in order to assure proper management of tumours producing gastroentero-pancreatic hormones.

Topics: Adenoma, Islet Cell; Carcinoid Tumor; Cholecystokinin; Gastric Inhibitory Polypeptide; Gastrins; Gastrointestinal Hormones; Glucagon; Glucagon-Like Peptides; Humans; Insulin; Intestinal Neoplasms; Motilin; Pancreatic Hormones; Pancreatic Neoplasms; Pancreatic Polypeptide; Radioimmunoassay; Secretin; Somatostatin; Substance P; Vasoactive Intestinal Peptide; Zollinger-Ellison Syndrome

Topics: Adenoma, Islet Cell; Glucagon; Humans; Islets of Langerhans; Pancreatic Neoplasms; Somatostatin; Vasoactive Intestinal Peptide; Zollinger-Ellison Syndrome

The value of ultrasonic scanning in the localization of hormone producing gastrointestinal tumours was assessed in 16 patients. Fifteen patients had Zollinger-Ellison syndrome and one patient had a VIP tumour. In four patients with Zollinger-Ellison syndrome ultrasonic scanning demonstrated a solid mass lesion of the head of the pancreas of 2.2.4 and 8 cm respectively. One of these diagnoses was verified by autopsy, one was also found by subsequent CT-scanning and angiography. In one patient ultrasonic scanning demonstrated a 3 cm tumour of the tail of the pancreas. At ultrasonically guided percutaneous fine needle biopsy tumour cells were aspirated. Biochemistry confirmed a VIP tumour. In 11 patients ultrasonic scanning was normal. By laparotomy in nine of these tumours of 1--3 cm were found in three patients 5.9 and 15 months after scanning and in two patients multiple 0.5 cm tumours within the duodenal wall were demonstrated one half and 9 months after ultrasound. In four patients tumours were not demonstrated by surgery. Two of the 11 patients were not operated upon, and the tumours have not been localized. The advantages of the complete atraumatic, rapid and relatively cheap ultrasound technique and the ability of an ultrasonically guided biopsy for the demonstration of malignancy justify ultrasonic scanning to be an initial method of investigation in the localization of hormone producing gastrointestinal tumours.

Topics: Evaluation Studies as Topic; Gastrointestinal Neoplasms; Humans; Pancreatic Neoplasms; Ultrasonography; Vasoactive Intestinal Peptide; Zollinger-Ellison Syndrome

Relationships between hormonal secretions from the GI tract and gastric functional and/or pathological abnormalities could be studied according to 2 main lines : 1) gastric secretory changes could be the main symptom of hormonal secretory tumors, i.e. acid hypersecretion in the Zollinger Ellison syndrome, acid hyposecretion in pancreatic cholera and in somatostatinoma. In these cases, hormonal hypersecretion is directly responsible for the functional disturbances and the related symptoms; 2) gastric pathological conditions are sometimes accompanied by changes in hormonal secretion, but the level of interdependence is variable : high blood gastrin is directly depending upon the atrophic gastritis in pernicious anemia; this mechanism was also suggested in case of gastric carcinoma. Concerning ulcer disease, numerous problems are unsolved in respect to blood gastrin (basal and stimulated) abnormalities, as well as somatostatin and GIP secretions.

Topics: Aged; Anemia, Pernicious; Cholera; Duodenal Ulcer; Gastric Inhibitory Polypeptide; Gastric Juice; Gastrins; Gastrointestinal Hormones; Gastrointestinal Neoplasms; Humans; Pancreatic Diseases; Pancreatic Neoplasms; Somatostatin; Stomach Diseases; Vasoactive Intestinal Peptide; Zollinger-Ellison Syndrome

A sensitive and specific radioimmunoassay for the detection of vasoactive intestinal peptide has been used to study patients with the watery diarrhea syndrome. In eleven patients the syndrome was associated with tumors, and plasma levels of vasoactive intestinal peptide were elevated. VIP levels returned towards normal in five treated patients coincident with amelioration of symptoms. Normal values were obtained in patinets with chronic pancreatitis, sprue, medullary carcinoma, Zollinger-Ellison Syndrome and laxative abuse. In six other patients with indistinguishable syndrome and no findings of tumor at laparotomy and autopsy, vasoactive intestinal peptide levels were normal. The results suggest that VIP may be the causative agent in patients with the watery diarrhea syndrome and tumors, but that an indistinguishable syndrome exists for which VIP is not the cause.

Topics: Diarrhea; Female; Gastrointestinal Hormones; Humans; Male; Middle Aged; Pancreatectomy; Pancreatic Neoplasms; Pancreatitis; Streptozocin; Syndrome; Vasoactive Intestinal Peptide; Zollinger-Ellison Syndrome

Topics: Brain Neoplasms; Calcitonin; Cholesterol; Diarrhea; Endocrine Glands; Gastrins; Gastrointestinal Hormones; Histamine Release; Humans; Hypercalcemia; Neoplasms; Nutrition Disorders; Pellagra; Peptides; Serum Albumin; Thyroid Hormones; Vasoactive Intestinal Peptide; Zollinger-Ellison Syndrome