FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W2613804234> ?p ?o ?g. }

• W2613804234 endingPage "225" @default.
• W2613804234 startingPage "223" @default.
• W2613804234 abstract "Case ReportsGlucagonoma, A Rare Cause of Obstructive Jaundice: Report of a Case Maurice Atiyeh, MD Mohammed Akhtar, MD Fayez Sandouk, and MD R. James St. HilaireMD Maurice Atiyeh From the Department of Medicine, King Faisal Specialist Hospital and Research Centre, Riyadh Search for more papers by this author , Mohammed Akhtar Address reprint requests and correspondence to Dr. Akhtar: Department of Pathology and Laboratory Medicine, King Faisal Specialist Hospital and Research Centre, P.O. Box 3354, Riyadh 11211, Saudi Arabia. From the Department of Pathology and Laboratory Medicine, King Faisal Specialist Hospital and Research Centre, Riyadh Search for more papers by this author , Fayez Sandouk From the Department of Medicine, King Faisal Specialist Hospital and Research Centre, Riyadh Search for more papers by this author , and R. James St. Hilaire From the Department of Medicine, King Faisal Specialist Hospital and Research Centre, Riyadh Search for more papers by this author Published Online:24 Apr 2019https://doi.org/10.5144/0256-4947.1988.223SectionsPDF ToolsAdd to favoritesDownload citationTrack citations ShareShare onFacebookTwitterLinked InRedditEmail AboutIntroductionGlucagonoma is a rare tumor originating from the alpha cells of the pancreatic islets. The diagnosis of this tumor is clinically suspected in the presence of the classical skin rash characterized by migratory necrotizing erythema. In addition, there is weight loss, stomatitis, and abnormal glucose tolerance test. With the introduction of radioimmune assay for serum levels of glucagon, more nonclassical cases are now being identified.1This article describes a patient with glucagonoma in whom the clinical presentation was in the form of obstructive jaundice and abdominal pain similar to that seen in association with an adenocarcinoma of the head of the pancreas. To our knowledge, this presentation has been reported only once in the literature.2CASE REPORTThe patient, a 53-year-old Syrian male, was admitted to a hospital in Syria because of obstructive jaundice. At laparotomy, a mass in the head of the pancreas was found. Examination of a biopsy specimen from the mass proved to be inconclusive, but the patient was diagnosed clinically as having adenocarcinoma of the pancreas that was considered to be inoperable. Eighteen months later the patient was admitted to the King Faisal Specialist Hospital and Research Centre. Clinical evaluation indicated that the patient had shown marked improvement during the last 18 months and had gained 10 kg. Routine biochemical profiles were within normal limits. The presence of a mass in the head of the pancreas was confirmed by CT scan and ultrasonography. At surgery, a frozen section of a needle biopsy of the tumor failed to confirm the diagnosis of adenocarcinoma of the pancreas. Permanent sections, however, revealed a tumor composed of small epithelial cells arranged in sheets and cords surrounded by dense fibrous stroma (Figure 1). Electron microscopy of the paraffin-embedded material revealed the presence of large numbers of neurosecretory granules within the tumor cells (Figure 2). A diagnosis of a pancreatic islet cell tumor was therefore suggested.Figure 1. Photomicrograph of an area from the needle biopsy taken from the pancreatic tumor. Clusters of small epithelial cells are surrounded by markedly dense stroma (hematoxylin and eosin stain, original magnification, × 100).Download FigureFigure 2. Electron micrograph showing tumor cells with several dense-core neurosecretory granules within the cytoplasm (uranyl acetate and lead citrate stain, original magnification, × 1200).Download FigureDeterminations of vasoactive intestinal polypeptide, somatostatin, pancreatic polypeptide, p-peptide, insulin, and gastrin blood levels were reported normal. However, the serum glucagon level was elevated, 980 pg/ml and 1,025 pg/ml (Bloom). His carcinoembryonic antigen was also elevated, 22 units/liter. After a ten-day course of streptozocin, a third laparotomy was performed, and the tumor was excised by performing a modified Whipple's procedure. The specimen was a 5×7×7-cm, firm, grayish-white mass in the head of the pancreas. The tumor bulged into the wall of the duodenum, but the mucosa was intact. Histologic examination confirmed the diagnosis of pancreatic islet cell tumor. The presence of glucagon within the tumor cells was demonstrated by immunoperoxidase staining. No tumor was noted outside the pancreas. Ultrasonography and CT scanning of the abdomen and chest showed no metastatic lesions.The patient has been followed for seven years without evidence of recurrence. He has been doing well on insulin and pancreatic enzyme supplements. Postsurgical and follow-up glucagon levels continue to be within normal limits.DISCUSSIONIn this case, an islet cell tumor, especially a glucagonoma, was not high on the list of possibilities because the patient did not have diabetes, weight loss, or any other finding, such as migratory necrolytic erythema, commonly present in patients with the glucagonoma syndrome. Absence of these features in a patient with glucagonoma is considered either due to lack of function by the tumor or due to the production of a larger-molecular-weight glucagon component. This has been reported to have a much less biologic activity than true glucagon.3–5 Diabetes mellitus or glucose intolerance is observed in the majority of patients (83%) with glucagonoma and is cured by complete resection of the tumor. Weight loss may be the result of the diabetes in addition to the catabolic activity of the glucagon. Anorexia is not a feature of the glucagonoma syndrome.6,7Migratory necrolytic erythema, a form of eczematous dermatitis, is the most characteristic clinical finding in glucagonoma.8 The skin lesion has a characteristic evolution from erythematous macule to larger fluid-filled vesicle followed by central necrosis and then by scaly eczematoid lesion with erythematous margin. Deficiencies of amino acids and essential fatty acids observed in patients with glucagonoma, presumably resulting from the metabolic action of the hormone, have been implicated in the pathogenesis of the skin rash. A marked improvement in the rash has been reported after peripheral intravenous infusion of 45.5 g of mixed amino acid daily for 6 days,9 while others have reported resolution of the rash after 2 weeks of intravenous administration of dextrose in saline.10Determination of the serum level of glucagon is essential to the clinical diagnosis of glucagonoma. The level in a glucagonoma should exceed 500 pg/ml,1,2 greater than three times the upper limit of normal. Elevation of serum levels of glucagon may also be seen in a variety of other conditions including diabetes mellitus, burns, renal failure, cirrhosis, bacteremia, and pheochromocytoma syndrome. In these cases, however, the glucagon level does not exceed three times the upper limit of normal.Glucagonoma, like other pancreatic islet cell tumors, is slow-growing, but metastases are often present at the time of diagnosis.7 The tumor arises most frequently in the tail and less commonly in the body of the pancreas. This distribution of the tumor conforms to the apparent distribution of alpha cells in normal pancreas.11 The tumor in our patient is unusual because of its occurrence in the head of the pancreas, a location that led to obstructive jaundice which resulted in an erroneous diagnosis of adenocarcinoma of the head of the pancreas.Glucagonoma without metastasis, as in this patient, is resectable with a high cure rate.6 Even in the presence of metastasis, surgical debulking by partial pancreatectomy can result in a better survival and clinical improvement.12 Chemotherapy with streptozocin or dimethyl triazenoimidazole carboxamide (DTIC) often results in regression of the tumor and symptomatic improvement.13In summary, this case of glucagonoma presented clinically as obstructive jaundice and without the usual symptoms of glucagonoma syndrome. This unusual clinical presentation caused difficulty in establishing a diagnosis which resulted in considerable delay in instituting a definite therapy. This case, we hope, will increase awareness among physicians that pancreatic adenoma on occasion may present with obstructive jaundice and thus mimic clinically adenocarcinoma of the head of the pancreas.ARTICLE REFERENCES:1. Friesem SR. Tumors of the endocrine pancreas . N Engl J Med. 1982; 306(10):580–90. Google Scholar2. Di Bisceglie AM, Segal I, Mannell A, Posen J. Pancreatic endocrine tumor presenting with obstructive jaundice . Am J Gastroenterol. 1984; 79(1):43–5. Google Scholar3. Weir GC, Norton ES, Aoki TT, et al.. Secretion by glucagonomas of a possible glucagon precursor . J Clin Invest. 1977; 59(2):325–30. Google Scholar4. Recant L, Perrino PV, Bhathena SJ, et al.. Plasma immunoreactive glucagon fractions in four cases of glucagonoma: increased “large glucagonimmunoreactivity.” . Diabetolgia. 1976; 12(4):319–26. Google Scholar5. Palmer JP, Werner PL, Benson JW, Ensinck JW. Dominant inheritance of large molecular weight immunoreactive glucagon . J Clin Invest. 1978; 61(3):763–9. Google Scholar6. Leichter BS. Clinical and metabolic aspects of glucagonoma . Medicine. 1980; 59(2):100–13. Google Scholar7. Higgins GA, Recant L, Fishman AB. The glucagonoma syndrome: surgically curable diabetes . Am J Surg. 1979; 137(1):142–8. Google Scholar8. Kahan RS, Perez-Figardo RA, Nuemanis A. Necrolytic migratory erythema: distinctive dermatosis of the glucagonoma syndrome . Arch Dermatol. 1977; 113(6):792–7. Google Scholar9. Norton JA, Kahn CR, Schiebinger R, et al.. Amino acid deficiency and the skin rash associated with glucagonoma . Ann Intern Med. 1979; 91(2):213–5. Google Scholar10. Marynick SP, Fagadau WR, Duncan LA. Malignant glucagonoma syndrome: response to chemotherapy . Ann Intern Med. 1980; 93(3)453–4. Google Scholar11. Orci L, Baeteus D, Ravazzola M, et al.. Pancreatic polypeptide and glucagon: non-random distribution in pancreatic islets . Life Sci. 1976; 19:1811. Google Scholar12. Montenegro F, Lawrence GD, Macon W, Pass C. Metastatic glucagonoma: improvement after surgical debulking . Am J Surg. 1980; 139(3):424–7. Google Scholar13. Kessinger A, Lemon HM, Foley JF. The glucagonoma syndrome and its management . J Surg Oncol. 1977; 9(5):419–24. Google Scholar Previous article Next article FiguresReferencesRelatedDetails Volume 8, Issue 3May 1988 Metrics History Accepted29 March 1987Published online24 April 2019 InformationCopyright © 1988, Annals of Saudi MedicinePDF download" @default.
• W2613804234 created "2017-05-19" @default.
• W2613804234 creator A5009947010 @default.
• W2613804234 creator A5048231349 @default.
• W2613804234 creator A5055694952 @default.
• W2613804234 creator A5088840363 @default.
• W2613804234 date "1988-05-01" @default.
• W2613804234 modified "2023-10-18" @default.
• W2613804234 title "Glucagonoma, A Rare Cause of Obstructive Jaundice: Report of a Case" @default.
• W2613804234 cites W1568192450 @default.
• W2613804234 cites W1964039793 @default.
• W2613804234 cites W1967327276 @default.
• W2613804234 cites W1983400139 @default.
• W2613804234 cites W1989523719 @default.
• W2613804234 cites W2031019303 @default.
• W2613804234 cites W2033632804 @default.
• W2613804234 cites W2038719145 @default.
• W2613804234 cites W2058793837 @default.
• W2613804234 cites W2069413832 @default.
• W2613804234 cites W2090459332 @default.
• W2613804234 cites W2132154644 @default.
• W2613804234 doi "https://doi.org/10.5144/0256-4947.1988.223" @default.
• W2613804234 hasPublicationYear "1988" @default.
• W2613804234 type Work @default.
• W2613804234 sameAs 2613804234 @default.
• W2613804234 citedByCount "0" @default.
• W2613804234 crossrefType "journal-article" @default.
• W2613804234 hasAuthorship W2613804234A5009947010 @default.
• W2613804234 hasAuthorship W2613804234A5048231349 @default.
• W2613804234 hasAuthorship W2613804234A5055694952 @default.
• W2613804234 hasAuthorship W2613804234A5088840363 @default.
• W2613804234 hasConcept C126322002 @default.
• W2613804234 hasConcept C16005928 @default.
• W2613804234 hasConcept C177713679 @default.
• W2613804234 hasConcept C2777127409 @default.
• W2613804234 hasConcept C2778764654 @default.
• W2613804234 hasConcept C2780053029 @default.
• W2613804234 hasConcept C3018539635 @default.
• W2613804234 hasConcept C61434518 @default.
• W2613804234 hasConcept C71924100 @default.
• W2613804234 hasConceptScore W2613804234C126322002 @default.
• W2613804234 hasConceptScore W2613804234C16005928 @default.
• W2613804234 hasConceptScore W2613804234C177713679 @default.
• W2613804234 hasConceptScore W2613804234C2777127409 @default.
• W2613804234 hasConceptScore W2613804234C2778764654 @default.
• W2613804234 hasConceptScore W2613804234C2780053029 @default.
• W2613804234 hasConceptScore W2613804234C3018539635 @default.
• W2613804234 hasConceptScore W2613804234C61434518 @default.
• W2613804234 hasConceptScore W2613804234C71924100 @default.
• W2613804234 hasIssue "3" @default.
• W2613804234 hasLocation W26138042341 @default.
• W2613804234 hasOpenAccess W2613804234 @default.
• W2613804234 hasPrimaryLocation W26138042341 @default.
• W2613804234 hasRelatedWork W2038711235 @default.
• W2613804234 hasRelatedWork W2070827075 @default.
• W2613804234 hasRelatedWork W2357629162 @default.
• W2613804234 hasRelatedWork W2360823297 @default.
• W2613804234 hasRelatedWork W2396654439 @default.
• W2613804234 hasRelatedWork W2430527564 @default.
• W2613804234 hasRelatedWork W2738981461 @default.
• W2613804234 hasRelatedWork W4234833016 @default.
• W2613804234 hasRelatedWork W4297184974 @default.
• W2613804234 hasRelatedWork W83854322 @default.
• W2613804234 hasVolume "8" @default.
• W2613804234 isParatext "false" @default.
• W2613804234 isRetracted "false" @default.
• W2613804234 magId "2613804234" @default.
• W2613804234 workType "article" @default.