gastrins and Malignant-Carcinoid-Syndrome

Several circulating or urinary tumour markers can be used for the diagnosis and follow-up of functioning and clinically non-functioning neuroendocrine tumours of the pancreatic islet cells and intestinal tract. Among the specific tumour markers are serotonin and its metabolites--e.g. 5-hydroxyindoleacetic acid (5-HIAA)--in carcinoid tumours and the carcinoid syndrome, insulin and its precursors or breakdown products in insulinoma, and gastrin in gastrinoma. Plasma vasointestinal polypeptide (VIP) determinations have been used in the diagnosis of VIPoma, plasma glucagon for glucagonoma, and serum somatostatin for somatostatinoma. Among the tumour-non-specific markers are: chromogranins, neuron-specific enolase (NSE), alpha-subunits of the glycoprotein hormones, catecholamines, pancreatic polypeptide (PP), ghrelin and adrenomedullin.

Topics: Biomarkers; Biomarkers, Tumor; Carcinoid Tumor; Gastrinoma; Gastrins; Humans; Insulin; Insulinoma; Malignant Carcinoid Syndrome; Neuroendocrine Tumors; Pancreatic Neoplasms; Serotonin

Topics: Adenoma, Islet Cell; Diagnosis, Differential; Diarrhea; Gastrins; Germany, West; Glucagon; Humans; Hyperinsulinism; Insulin; Insulin Secretion; Malignant Carcinoid Syndrome; Pancreatic Neoplasms; Syndrome; Zollinger-Ellison Syndrome

14 patients with metastatic endocrine gastro-entero-pancreatic carcinoma (6 patients with Carcinoid-syndrome, 3 with gastrinoma and 5 with non-functioning tumor) have been treated with Octreotide 3 x 200 micrograms/die plus Interferon-Alpha 3 x 5 Mio U/week after documented tumor progression during preceding Octreotide-monotherapy. 6 out of 14 patients responded favourable to the treatment: one patient with partial regression and 5 patients with stillstand of tumor growth. In only one patient initial tumor stillstand for 6 months was followed by tumor progression whereas in five patients a beneficial effect on tumor growth could be documented up to 34 months. Inhibition of tumor growth and tumor progression was not necessarily paralleled by respective changes in peripheral hormone levels. These results should initiate a controlled prospective study to prove the hypothesis that in patients with metastasized endocrine gastro-entero-pancreatic tumors the combination of Octreotide and Interferon-Alpha is superior to monotherapy with Octreotide or Interferon-Alpha and to identify those patients who respond to this combined therapy.

Topics: Biomarkers, Tumor; Combined Modality Therapy; Female; Gastrinoma; Gastrins; Gastrointestinal Neoplasms; Humans; Hydroxyindoleacetic Acid; Interferon alpha-2; Interferon-alpha; Liver Neoplasms; Male; Malignant Carcinoid Syndrome; Neoplasm Metastasis; Neuroendocrine Tumors; Octreotide; Pancreatic Neoplasms; Recombinant Proteins

The authors report the case of a patient with a typical carcinoid syndrome and a severe hypoglycemia due to hyperinsulinism. He was found to have an ileal carcinoid tumor with hepatic metastasis and no evidence of pancreatic insulinoma at surgery and autopsy. By assaying serotonin and insulin in the tumor and in the supernatants of the culture derived from hepatic metastasis, the authors have been able to show that both hormones were produced by the carcinoid tissue. Cultured cells also synthesized minute amounts of gastrin and thyrocalcitonin.

Topics: Blood Glucose; Calcitonin; Carcinoid Tumor; Cells, Cultured; Cytoplasmic Granules; Gastrins; Humans; Ileal Neoplasms; Insulin; Insulin Secretion; Liver Neoplasms; Male; Malignant Carcinoid Syndrome; Microscopy, Electron; Middle Aged; Serotonin

Somatostatin (4 micrograms/min, cyclic form) was infused for 24 h on six occasions in four patients with carcinoid ileal tumor and multiple liver metastases. During infusion, spontaneous flushes and/or diarrhea were markedly reduced with a return to the pretreatment pattern on the next day. Urinary 5-hydroxyindole acetic acid excretion and plasma gastrin were not significantly changed, whereas plasma insulin presented the expected decrease and rebound. These results confirm that carcinoid symptoms are somatostatin-sensitive but do not provide evidence that serotonin or gastrin play a major role in inducing these symptoms.

Topics: Aged; Diarrhea; Female; Gastrins; Humans; Hydroxyindoleacetic Acid; Insulin; Male; Malignant Carcinoid Syndrome; Middle Aged; Skin; Somatostatin

Conventional or electron microscopy can contribute significantly to the diagnosis of pancreatic endocrine tumours. These techniques, however, are of limited value for the classification of the tumours, which should take both clinical and morphological findings into account. In this paper a classification based on both the clinical features and on the content of peptide hormone-producing cells in the tumours is presented.

Topics: Adenoma, Islet Cell; Cushing Syndrome; Gastrins; Glucagon; Humans; Malignant Carcinoid Syndrome; Pancreatic Neoplasms; Somatostatin; Zollinger-Ellison Syndrome

Topics: Gastrins; Humans; Malignant Carcinoid Syndrome; Somatostatin

Topics: Adrenal Gland Diseases; Endocrine System Diseases; Erythrocytes; Female; Gastrins; Gynecomastia; Hormones, Ectopic; Humans; Hypercalcemia; Hyperplasia; Hyperthyroidism; Hypoglycemia; Male; Malignant Carcinoid Syndrome; Metabolic Diseases; Neoplasms; Neurologic Manifestations; Polycythemia; Prognosis; Puberty, Precocious; Vasopressins