Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0011849 (diabetes)
 277,896 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors report two cases of glucagonoma, a rare endocrine tumor of the pancreas, and describe the data currently found in literature. Glucagonoma is a single and usually large tumor, which develops in the alpha cells of the islets of Langerhans and evolves slowly. The combination of characteristic skin lesions, diabetes and weight loss should lead to searching for hyperglucagonemia and for the pancreatic tumor. The diagnosis is usually made rather late, average evolution is five years before diagnosis when it is detected. Imaging, in particular ultrasound and computed tomography (CT), proves to be necessary for the positive diagnosis of glucagonoma as it localizes the pancreatic mass and plays a role in local assessment, thus providing guidance for surgery. The role of imaging is also fundamental for the detection of metastases, which are the only sign of malignancy as no criterion of benignity is found for this tumor.
 ...
PMID:[Diagnosis of glucagonoma. Value of scanning, echography and arteriography. Apropos of 2 cases and a review of the literature]. 255 85

We report a case of pancreatic tumour metastatic to the liver in a patient with insulin-treated diabetes, anaemia, cheilitis, necrolytic migratory erythema, hypokalemia and chronic watery diarrhea, a picture suggesting combined glucagonoma and VIPoma syndromes. Immunocytochemistry of a biopsied hepatic metastatic nodule revealed both glucagon and vasoactive intestinal peptide (VIP) positive cells. Increased plasma glucagon and VIP levels were detected (values of 900 pmol/l and 277 pmol/l respectively). This is the first reported case showing not only immunocytochemical, but also clinical evidence of the combined secretion of these hormones.
 ...
PMID:A combined glucagonoma and VIPoma syndrome. First pathologic and clinical report. 284 62

A long-acting somatostatin analog, SMS 201-995, is now available to treat the hormonal manifestations of islet cell tumors. We report its use in a patient with a metastatic glucagonoma refractory to conventional therapy. This patient, who was severely disabled by the rash of necrolytic migratory erythema and brittle diabetes mellitus, allowed us to evaluate the therapeutic efficacy of SMS 201-995 and to gain insight into the origin of the rash. SMS 201-995 was administered subcutaneously (.05 mg twice a day). The rash improved markedly within 48 hours and was completely resolved within 1 week of treatment. Insulin requirements decreased from 90 U/day to zero during the first week of treatment. Corresponding to improvement in clinical symptoms circulating glucagon levels showed a marked decrease. There was no substantial change in plasma or urinary levels of zinc or in plasma amino acid levels. When SMS 201-995 was stopped, the rash recurred within 36 hours and it improved within 48 hours of readministration. The rash and diabetes have remained well controlled during 8 months of therapy but no change in tumor size has been seen on CT scan. The rapid changes in the rash related to the administration of SMS 201-995 indicate that the pathogenesis of necrolytic migratory erythema is probably due to circulating hyperglucagonemia or some other hormonal substance produced by the tumor.
 ...
PMID:Use of a somatostatin analog (SMS 201-995) in the glucagonoma syndrome. 287

The glucagonoma syndrome is characterized by a necrolytic migratory erythematous rash, angular stomatitis, painful glossitis, a normochromic normocytic anemia, mild diabetes mellitus, weight loss, a tendency to thrombosis, and neuropsychiatric disturbances. The diagnosis is made by finding a high plasma glucagon concentration in the absence of any other cause, such as renal failure or severe stress. A pancreatic alpha-cell tumor can be identified and stained by immunocytochemistry with glucagon antibodies. Optimal treatment is surgical removal, but approximately 50 percent of the tumors have metastasized by the time of diagnosis. Since the tumor is slow-growing, remission can be obtained by hepatic artery embolization to shrink hepatic secondaries or by shrinkage, in about 10 percent of patients, with the combination chemotherapeutic regimen of 5-fluorouracil and streptozotocin. The rash frequently responds to administration of zinc, a high-protein diet, and control of the diabetes with insulin. Alongside the alpha cell in the islets of Langerhans is the D-cell, which produces somatostatin and may well act physiologically as a paracrine inhibitor of glucagon release. A newly developed, long-acting somatostatin analogue, SMS 201-995, which the patient can self-administer as a subcutaneous injection, has proven effective in suppressing glucagon secretion from glucagonomas and, in some cases, causing remission of clinical symptoms.
 ...
PMID:Glucagonoma syndrome. 288 77

The embryogenesis of the pancreas suggests the existence of a common stem cell progenitor of the four islet cell types (insulin, glucagon, somatostatin, and pancreatic polypeptide). We investigated whether neoplastic islet tumors express multiple hormone-specific cellular phenotypes of the islets. By analyses of RNA transcripts and immunoreactive peptides in four human insulinomas and one glucagonoma, we found that the insulin, somatostatin, and glucagon genes were coexpressed in all tumors. The expression of the three hormone genes in a lymph node metastasis of a glucagonoma reduced the possibility that contamination of tumor tissue by normal islets occurred. These observations lend further support to the hypothesis of the multipotentiality of neoplastic islet cells for the expression of genes encoding several different islet hormones.
Diabetes 1988 Dec
PMID:Expression of peptide hormone genes in human islet cell tumors. 290 36

Seven patients with clinical features of the glucagonoma syndrome, including the characteristic rash, diabetes mellitus, and weight loss, were examined by CT. Computed tomography demonstrated a primary pancreatic tumor in all patients, and, in four, hepatic metastases were identified. The primary tumors, relatively large and solid in nature, varied in size from 2.5 to 6 cm in maximum diameter. The tumor was found in the tail of the pancreas in three patients and in the head of the pancreas in four. No obliteration of adjacent perivascular or peripancreatic fat planes was observed in any patient. Calcification was present in the primary tumor in three patients and in the hepatic metastases in one. All tumors that were studied angiographically were found to be hypervascular. In contrast to insulinomas, which are frequently quite small when clinically diagnosed, glucagonomas appear to attain considerable size prior to being clinically apparent. Thus, we conclude that CT ought to become the mainstay in the identification, localization, and staging of these tumors.
 ...
PMID:CT evaluation of glucagonomas. 298 9

A 63 year old man presented with features of the glucagonoma syndrome, that is thromboembolic disease, weight loss, raised sedimentation rate, diabetes mellitus, hypoproteinaemia and reduced plasma amino acid levels, but without necrolytic migratory erythema. The plasma glucagon level was raised and the tumour was demonstrated by abdominal CT scan. Immunofluorescent studies of the resected tumour confirmed the diagnosis. The normal tissue zinc status supports the view that necrolytic migratory erythema is related to zinc deficiency.
 ...
PMID:Glucagonoma without cutaneous manifestations. 299 32

A case of a 58-year-old woman with an unusual variant of malignant islet-cell tumor showing oncocytic features is described. Using the light microscopy technique, the tumor appeared comprised of solid nests of uniform cells with abundant, eosinophilic cytoplasm and round nuclei with granular chromatin. Ultrastructurally, the cells contained numerous abnormal mitochondria, dilated rough endoplasmic reticulum, and scattered dense-core neurosecretory granules, often associated with cytoplasmic filaments. Tumor cells were focally immunoreactive for insulin, glucagon, and somatostatin and diffusely immunoreactive for alpha 1-antitrypsin as assayed by the avidin--biotin technique. The tumor was immunonegative for human chorionic gonadotropin, gastrin, adrenocorticotropic hormone, and serotonin. The patient exhibited some of the clinical features associated with glucagonoma syndrome, including diabetes mellitus and chronic diarrhea. The tumor behaved in a malignant fashion, with widespread lymphatic involvement and bony metastases at the time of presentation. This report of an oncocytic islet-cell carcinoma supports the concept of oncocytic differentiation in islet-cell tumors in a fashion analagous to oncocytic carcinoids.
 ...
PMID:Functioning oncocytic islet-cell carcinoma. Report of a case with electron-microscopic and immunohistochemical confirmation. 300 44

The secretory response and immunoreactive heterogeneity of glucagon was investigated in a patient with glucagonoma syndrome. After glucose administration, abnormal insulin release accompanied by glucose intolerance were observed, whereas the high glucagon circulating levels were only partially blocked after glucose or somatostatin infusion. Chromatographic fractionation of plasma samples, before and after arginine administration showed that most of the immunoreactivity eluted as true glucagon. Furthermore, when aliquots of the tumor extracts were fractionated by column chromatography or by polyacrylamide gel electrophoresis, most of the immunoreactivity eluted in the 3,500 molecular weight peak. In contrast with previous reports, our results indicate that neoplasia A cells can also manufacture and release into the bloodstream great amounts of genuine glucagon rather than larger glucagon immunoreactive forms. In spite of such findings, in this patient neither diabetes nor hyperglycemia were present.
 ...
PMID:Secretory response and immunochemical heterogeneity of glucagon in plasma and tumor extracts of a patient with glucagonoma. 300 53

An autopsy confirmed, primary malignant APUDoma of the liver is described. Immunohistochemical methods demonstrated the presence of glucagon in some 20% of tumor cells. Clinically the 66 year old patient had presented with an abortive form of the glucagonoma syndrome with diabetes mellitus, anemia and profound weight loss.
 ...
PMID:[Unusual abdominal apudomas. II. Immunohistochemical detection of glucagon in a primary apudoma of the liver]. 391 90


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>