vasoactive-intestinal-peptide and Endocrine-System-Diseases

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

Information concerning GEP hormones has progressively advanced since the initial discovery of a GEP hormone, secretin, in 1902. Studies in this area flourished with the advent of radioimmunoassay, and have provided an understanding of the secretion, regulation, metabolic actions, and role in certain diseases of major GEP hormones. Measurement of GEP hormones has achieved importance in clinical medicine and allowed understanding of the pathophysiology of several clinical disorders. The decade to come should witness additional advances in this rapidly expanding field.

Topics: Chemical Phenomena; Chemistry; Cholecystokinin; Diabetes Mellitus; Diarrhea; Endocrine System Diseases; Gastric Inhibitory Polypeptide; Gastrins; Gastrointestinal Hormones; Glucagon; Humans; Hypoglycemia; Motilin; Neoplasms; Neurotensin; Pancreatic Polypeptide; Peptic Ulcer; Secretin; Skin Diseases; Somatostatin; Substance P; Vasoactive Intestinal Peptide

A 31-year-old patient with a clinical picture of obstructive jaundice had surgical treatment, and a primary carcinoid of the ampulla of Vater (VA) was found. The tumor was studied with light microscopy, immunohistochemistry, and electron microscopy. The neoplasm had histopathologic and cytopathologic features similar to those encountered in typical neuroendocrine neoplasms. It is interesting that immunohistochemical techniques disclosed the presence of vasointestinal polypeptide, cholecystokinin, and bombesin; however, unlike most neuroendocrine neoplasms arising in VA, no somatostatin-immunoreactive cells were found.

Topics: Adult; Ampulla of Vater; Bombesin; Cholecystokinin; Common Bile Duct Neoplasms; Endocrine System Diseases; Humans; Male; Microscopy, Electron; Nervous System Neoplasms; Somatostatin; Vasoactive Intestinal Peptide

Peptide radioimmunoassay has become an important clinical and research tool in understanding the role of peptides in the pathophysiology of gut endocrine tumor syndromes. A gut peptide radioimmunoassay laboratory has been established for the diagnosis and clinical monitoring of endocrine tumors of the gastroenteropancreatic (GEP) system. Radioimmunoassay has enhanced our awareness that co-occurring peptide interactions may modify and ultimately influence the clinical expression of these tumors. Furthermore, it has helped develop a rationale for the use of prototype peptides such as somatostatin and its long-acting analogue Sandostatin (SMS 201-995) in the management of GEP tumors. This group's experience, as well as the experience of other investigators, is presented, and the clinical utility of peptide radioimmunoassay in the field of gut endocrinology is demonstrated.

Topics: Adult; Endocrine System Diseases; Female; Gastrointestinal Hormones; Gastrointestinal Neoplasms; Humans; Male; Middle Aged; Octreotide; Pancreatic Neoplasms; Peptides; Radioimmunoassay; Somatostatin; Vasoactive Intestinal Peptide

Topics: Animals; Diabetes Mellitus; Endocrine System Diseases; Gastrins; Heart; Hormones; Humans; Insulin; Insulin Secretion; Islets of Langerhans; Ketone Bodies; Myocardium; Periodicity; Rats; Receptor, Insulin; Receptors, Adrenergic, beta; Receptors, Muscarinic; Somatostatin; Vasoactive Intestinal Peptide

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

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

Topics: Achlorhydria; APUD Cells; Apudoma; Carcinoid Tumor; Carcinoma; Cholecystokinin; Diarrhea; Endocrine System Diseases; Humans; Hypokalemia; Malignant Carcinoid Syndrome; Neoplasms; Pancreatic Diseases; Paraganglioma; Paraneoplastic Endocrine Syndromes; Peptides; Prostaglandins E; Somatostatin; Syndrome; Thyroid Neoplasms; Vasoactive Intestinal Peptide

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

Topics: Animals; Congresses as Topic; Endocrine System Diseases; Gastric Inhibitory Polypeptide; Gastric Juice; Gastrins; Gastrointestinal Hormones; Gastrointestinal Motility; Glucagon-Like Peptides; Histamine; Humans; Insulin; Insulin Secretion; Motilin; Neoplasms; Neurotransmitter Agents; Pancreatic Hormones; Pancreatic Juice; Pancreatic Polypeptide; Secretin; Somatostatin; Vasoactive Intestinal Peptide