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Glucagonoma and Its Management

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Glucagon III

Part of the book series: Handbook of Experimental Pharmacology ((HEP,volume 123))

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Abstract

Glucagonomas are considered to be among the rarest of the islet-cell tumors, with approximately 200 cases reported to date, even though they may be underdiagnosed clinical entities (Edey et al. 1990). Because of their uncommon occurrence, recognition is often delayed until metastases have developed. This occurs despite the fact that glucagonomas elicit a well-defined clinical syndrome that comprises distinctive dermatosis (“necrolytic migratory erythema”), diabetes, diarrhea, weight loss, anaemia and, more rarely, dementia. A detailed description of the glucagonoma syndrome has been previously reported (Mallinson et al. 1974; Stacpoole 1981; Montenegrorodas and Samaan 1981; Luyckx and Lefebvre 1981; Guillausseau et al. 1982) and particularly in Chap. 43 of the previous volume in this series, Glucagon II (Wood et al. 1983). Since then, several reviews have been devoted to this syndrome (Parker et al. 1984; Holst 1985; Bloom and Polak 1987; Hashiziume et al. 1988; Somers and De Vroede 1988; Boden 1989; Jockenhövel and Reinwein 1992).

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References

  • Ajani JA, Carrasco CH, Charnsangavej C, Samaan NA, Levin B, Wallace S (1988) Islet cell tumors metastatic to the liver: effective palliation by sequential hepatic artery embolization. Ann Intern Med 108: 340–344

    PubMed  CAS  Google Scholar 

  • Ajani JA, Levin B, Wallace S (1989) Systemic and regional therapy of advanced islet cell tumors. Gastroenterol Clin North Am 18: 923–930

    PubMed  CAS  Google Scholar 

  • Ajani JA, Carrasco CH, Samaan NA, Wallace S (1991) Therapeutic options for patients with advanced islet cell and carcinoid tumors. Reg Cancer Treat 3: 235–242

    Google Scholar 

  • Allison DJ, Jordan H, Hennessy O (1985) Therapeutic embolization of the hepatic artery. A review of 75 procedures. Lancet I: 595–599

    Google Scholar 

  • Altimari AF, Bhoopalam N, O’Dorsio T, Lange CL, Sandberg L, Prinz RA (1986) Use of a somatostatin analog (SMS 201–995) in the glucagonoma syndrome. Surgery 100: 989–996

    PubMed  CAS  Google Scholar 

  • Altimari AF, Badrinath K, Reisel HJ, Prinz RA (1987) DTIC therapy in patients with malignant intra-abdominal neuroendocrine tumors. Surgery 102: 1009–1017

    PubMed  CAS  Google Scholar 

  • Arnaud A, Fetissof F, Lorette G, Reigner J, Bertrand G, Lecomte P (1992) Le syndrome du glucagonome: trois nouveaux cas. Rev Med Interne 13: 103–108

    Article  PubMed  CAS  Google Scholar 

  • Arnold R, Neuhaus C, Benning R, Schwerk WB, Trautmann ME, Joseph K, Bruns C (1993) Somatostatin analog sandostatin and inhibition of tumor growth in patients with metastatic endocrine gastroenteropancreatic tumors. World J Surg 17: 511–519

    Article  PubMed  CAS  Google Scholar 

  • Assaad SN, Carrasco CH, Vassilopoulou-Sellin R, Samaan NA (1987) Glucagonoma syndrome: rapid response following arterial embolization of glucagonoma metastatic to the liver. Am J Med 82: 533–535

    Article  PubMed  CAS  Google Scholar 

  • Blanchin M, James-Deidier A, Chaumet-Riffaud PD, Chayvialle J-A (1992) Utilisation de l’octréotide dans les tumeurs endocrines digestives. Etude française multicentrique. Presse Med 21: 697–702

    PubMed  CAS  Google Scholar 

  • Bloom SR, Polak JR (1987) Glucagonoma syndrome. Am J Med 82 (Suppl 5B): 25–36

    Article  PubMed  CAS  Google Scholar 

  • Boden G (1989) Glucagonomas and insulinomas. Gastroenterol Clin North Am 18: 831–845

    PubMed  CAS  Google Scholar 

  • Boden G, Ryan IG, Eisenschmid BL, Shelmet JJ, Owen OE (1986) Treatment of inoperable glucagonoma with the long acting somatostatin analogue SMS 201–995. N Engl J Med 314: 1686–1689

    Article  PubMed  CAS  Google Scholar 

  • Breatnach ES, Han SY, Rahatzad MT, Stanley RJ (1985) CT evaluation of glucagonomas. J Comput Assist Tomogr 9: 25–29

    Article  PubMed  CAS  Google Scholar 

  • Broder LE, Carter SK (1973) Pancreatic islet cell carcinoma. Results of therapy with streptozotocin in 52 patients. Ann Intern Med 79: 108–118

    PubMed  CAS  Google Scholar 

  • Bukowski RM, Tangen C, Lee R, Macdonald JS, Einstein AB Jr, Peterson R, Fleming TR (1992) Phase II trial of chlorozotocin and fluorouracil in islet cell carcinoma: a southwest oncology group study. J Clin Oncol 10: 1914–1918

    PubMed  CAS  Google Scholar 

  • Carty SE, Jensen RT, Norton JA (1992) Prospective study of aggressive resection of metastatic pancreatic endocrine tumors. Surgery 112: 1024–1032

    PubMed  CAS  Google Scholar 

  • Ch’ng JLC, Anderson JV, Williams SJ, Carr DH, Bloom SR (1986) Remission of symptoms during long term treatment of metastatic pancreatic endocrine tumours with long acting somatostatin analogue. Br Med J 292: 981–982

    Article  Google Scholar 

  • Debas HT, Gittes G (1993) Somatostatin analogue therapy in functioning neuroendocrine gut tumors. Digestion 54 [Suppl] 1: 68–71

    Google Scholar 

  • Edney JA, Hofmann S, Thompson JS, Kessinger A (1990) Glucagonoma syndrome is an underdiagnosed clinical entity. Am J Surg 160: 625–629

    Article  PubMed  CAS  Google Scholar 

  • Eriksson B, Öberg K, Alm G, Karlsson A, Lundqvist G, Andersson T, Wilander E, Wide L (1986) Treatment of malignant endocrine pancreatic tumours with human leucocyte interferon. Lancet II: 1307–1309

    Article  Google Scholar 

  • Fraker DL, Norton JA (1989) The role of surgery in the management of islet cell tumors. Gastroenterol Clin North Am 18: 805–830

    PubMed  CAS  Google Scholar 

  • Freimann J, Pazdur R (1990) Hepatic arterial embolization for treatment of glucagonomas: antineoplastic and palliative benefits. Am J Clin Oncol 13: 271–275

    Article  PubMed  CAS  Google Scholar 

  • Friesen SR (1987) Update on the diagnosis and treatment of rare neuroendocrine tumors. Surg Clin North Am 67: 379–393

    PubMed  CAS  Google Scholar 

  • Fujita J, Seino Y, Ishida H, Taminato T, Matsukura S, Horio T, Imamura S, Naito A, Tobe T, Takahashi K, Midorikawa O, Imura H (1986) A functional study of a case of glucagonoma exhibiting typical glucagonoma syndrome. Cancer 57: 860–865

    Article  PubMed  CAS  Google Scholar 

  • Glover JR, Shorvon PJ, Lees WR (1992) Endoscopic ultrasound for localisation of islet cell tumours. Gut 33: 108–110

    Article  PubMed  CAS  Google Scholar 

  • Grama D, Eriksson B, Martensson H, Cedermark B, Ahren B, Kristoffersson A, Rastad J, Oberg K, Akerström G (1992) Clinical characteristics, treatment and survival in patients with pancreatic tumors causing hormonal syndromes. World J Surg 16: 632–639

    Article  PubMed  CAS  Google Scholar 

  • Grant CS (1993) Surgical management of malignant islet cell tumors. World J Surg 17: 498–503

    Article  PubMed  CAS  Google Scholar 

  • Guillausseau P-J, Guillausseau C, Villet R, Kaloustian E, Valleur P, Hautefeuille P, Lubetzki J (1982) Les glucagonomes. Aspects cliniques, biologiques, anatomopathologiques et thérapeutiques (revue générale de 130 cas). Gastroenterol Clin Biol 6: 1029–1041

    PubMed  CAS  Google Scholar 

  • Hallengren B, Dymling JF, Manhem P, Tennvall L, Tibblin S (1983) Unsuccessful DTIC treatment of a patient with glucagonoma syndrome. Acta Med Scand 213: 317–318

    Article  PubMed  CAS  Google Scholar 

  • Hashizume T, Kiryu H, Noda K, Kano T, Nakano R (1988) Glucagonoma syndrome. J Am Acad Dermatol 19: 377–383

    Article  PubMed  CAS  Google Scholar 

  • Hercot O, Legmann P, Humbert M, Sibert A, Somveille E, Picard C, Mignon M, Benacerraf R (1989) Diagnostic du glucagonome. Intérêt du scanner, de l’échographie et de l’artériographie. A propos de deux observations et revue de la littérature. J Radiol 70: 309–316

    CAS  Google Scholar 

  • Holmes A, Kilpatrick C, Proietto J, Green MD (1991) Reversal of a neurologic paraneoplastic syndrome with octreotide (Sandostatin) in a patient with glucagonoma. Am J Med 91: 434–436

    Article  PubMed  CAS  Google Scholar 

  • Holst JJ (1985) Glucagon-producing tumors. In: Cohen S, Soloway RD (eds) Hormone-producing tumors of the gastrointestinal tract. Contemporary issues in gastroenteroloyg, vol 5. Churchill Livingstone, New York, pp 57–84

    Google Scholar 

  • Ingemansson S, Holst J, Larsson LI, Lunderquist A (1977) Localization of glucagonomas by catheterization of the pancreatic veins and with glucagon assay. Surg Gynecol Obstet 145: 509–516

    PubMed  CAS  Google Scholar 

  • Jeanmougin M, Civatte J, Bonvalet D, Passa P, Verola O, Zylberait D (1988) Glucagonome métastatique. Rémission complète par la dacarbazine (Recul de 5 ans). Ann Dermatol Venereol 115: 833–838

    PubMed  CAS  Google Scholar 

  • Jensen RT, Norton JA (1995) Endocrine neoplasms of the pancreas. In: Yamada T (ed) Textbook of gastroenterology. Lippincott, Philadelphia, chap. 93, pp 2131–2160

    Google Scholar 

  • Jian R, Seyrig JA, Roche A, Modigliani R, Lenormand Y, Hautefeuille M (1984) Improvement of metastatic glucagonoma by hepatic artery embolization. Gastroenterology 87: 481–482

    PubMed  CAS  Google Scholar 

  • Jockenhövel F, Reinwein D (1992) Das Glukagonom: klinische Aspekte, Diagnostik und Therapie. Aktuel Endokrinol 13: 66–80

    Google Scholar 

  • Jockenhövel F, Lederbogen S, Olbricht T, Schmidt-Gayk H, Krenning EP, Lamberts SWJ, Reinwein D (1994) The long-acting somatostatin analogue octreotide alleviates symptoms by reducing posttranslational conversion or prepro-glucagon to glucagon in a patient with malignant glucagonoma, but does not prevent tumor growth. Clin Invest Med 72: 127–133

    Google Scholar 

  • Jones DV, Samaan NA, Sellin RV, Ajani JA (1992) Metastatic glucagonoma: clinical response to a combination of 5-fluorouracil and a-interferon. Am J Med 93: 348–349

    Article  PubMed  Google Scholar 

  • Kessinger A, Foley JF, Lemon HM (1983) Therapy of malignant APUD cell tumors. Effectiveness of DTIC. Cancer 51: 790–794

    Article  PubMed  CAS  Google Scholar 

  • Khandekar JD, Sriratana P (1986) Response of glucagonoma syndrome to lomustine. Cancer Treat Rep 70: 433–434

    PubMed  CAS  Google Scholar 

  • Krenning EP, Kooij PPM, Bakker WH, Breeman WAP, Postema PTE, Kwekkeboom DJ, Oei HY, de Jong M, Visser TJ, Reijs AEM, Lamberts SWJ (1994) Radiotherapy with a radiolabeled somatostatin analogue, (1111n-DTPA-D-Phe1)octreotide. A case history. Ann NY Acad Sci 733: 496–506

    Article  CAS  Google Scholar 

  • Krudy AG, Doppman LJ, Jensen RT, Norton JA, Cohen MJ, Shawker TH, Gardner JD, McArthur K, Gorden P (1984) Localization of islet tumors by dynamic CT: comparison with plain CT, arteriography, sonography and venous sampling. Am J Roentgenology 143: 585–589

    CAS  Google Scholar 

  • Kurose T, Seino Y, Ishida H, Fujita J, Taminato T, Matsukura M, Imura H (1984) Successful treatment of metastatic glucagonoma with dacarbazine. Lancet 1: 621–622

    Article  PubMed  CAS  Google Scholar 

  • Kvols LK, Buck M (1987) Chemotherapy of endocrine malignancies: a review. Semin Oncol 14: 343–353

    PubMed  CAS  Google Scholar 

  • Lamberts SWJ, Bakker WH, Reubi J-C, Krenning EP (1990) Somatostatin-receptor imaging in the localization of endocrine tumors. N Engl J Med 323: 1246–1249

    Article  PubMed  CAS  Google Scholar 

  • Lamberts SWJ, Krenning EP, Reubi J-C (1991) The role of somatostatin and its analogs in the diagnosis and treatment of tumors. Endocr Rev 12: 450–482

    Article  PubMed  CAS  Google Scholar 

  • Lamberts SWJ, Chayvialle J-A, Krenning EP (1993) The visualization of gastroenteropancreatic endocrine tumors. Digestion 54 [Suppl 1]: 92–97

    Article  PubMed  Google Scholar 

  • Lax E, Leibovici V, Fields SI, Gordon RL (1986) Neglected radiologie signs of the glucagonoma syndrome. Diag Imag Clin Med 55: 321–326

    CAS  Google Scholar 

  • Lokich J, Bothe A, O’Hara C, Federman M (1987) Metastatic islet cell tumor with ACTH, gastrin, and glucagon secretion. Clinical and pathologic studies with multiple therapies. Cancer 59: 2053–2058

    CAS  Google Scholar 

  • Luyckx AS, Lefèbvre PJ (1981) Les glucagonomes. Diabete Metab 7: 289–300

    PubMed  CAS  Google Scholar 

  • Mallinson CN, Bloom SR, Warin AP, Salmon PR, Cox B (1974) A glucagonoma syndrome. Lancet II: 1–5

    Google Scholar 

  • Maton PN (1993) Use of octreotide acetate for control of symptoms in patients with islet cell tumors. World J Surg 17: 504–510

    Article  PubMed  CAS  Google Scholar 

  • Maton PN, Gardner JD, Jensen RT (1989) Use of long-acting somatostatin analog SMS 201–995 in patients with pancreatic islet cell tumors. Dig Dis Sci 34 [Suppl]: 285–395

    Google Scholar 

  • McEntee GP, Nagorney DM, Kvols LK, Moertel CG, Grant CS (1990) Cytoreductive hepatic surgery for neuroendocrine tumors. Surgery 108: 1091–1096

    PubMed  CAS  Google Scholar 

  • Mignon M, Jensen RT (eds) (1995) Endocrine tumors of the pancreas: recent advances in research and management. Karger, Basel

    Google Scholar 

  • Moattari AR, Cho K, Vinik AI (1990) Somatostatin analogue in treatment of coexisting glucagonoma and pancreatic pseudocyst: dissociation of responses. Surgery 108: 581–587

    PubMed  CAS  Google Scholar 

  • Modlin IM, Lewis JJ, Ahlman H, Bilchik AJ, Kumar RR (1993) Management of unresectable malignant endocrine tumors of the pancreas. Surg Gynecol Obstet 176: 507–518

    PubMed  CAS  Google Scholar 

  • Moertel CG, Kvols LK, O’Connell MJ, Rubin J (1991) Treatment of neuroendocrine carcinomas with combined etoposide and cisplatin. Cancer 68: 227–232

    Article  PubMed  CAS  Google Scholar 

  • Moertel CG, Lefkopoulo M, Lipsitz S, Hahn RG, Klaassen D (1992) Streptozotocindoxorubucin, streptozotocin-fluorouracil, or chlorozotocin in the treatment of advanced islet-cell carcinoma. N Engl J Med 326: 519–523

    Article  PubMed  CAS  Google Scholar 

  • Montenegro-Rodas F, Samaan NA (1981) Glucagonoma tumors and syndrome. Curr Probi Cancer 6: 1–54

    Article  CAS  Google Scholar 

  • Nesovic M, Ciric J, Radojkovic S, Zarkovic M, Durovic M (1992) Improvement of metastatic endocrine tumors of the pancreas by hepatic artery chemoembolization. J Endocrinol Invest 15: 543–547

    PubMed  CAS  Google Scholar 

  • Nocaudie-Calzada M, Huglo D, Deveaux M, Carnaille B, Proye Ch, Marchandise X (1994) Iodine-123-Tyr-3-octreotide uptake in pancreatic endocrine tumors and in carcinoids in relation to hormonal inhibition by octreotide. J Nucl Med 35: 57–62

    PubMed  CAS  Google Scholar 

  • Obeg K, Eriksson B (1989) Medical treatment of neuroendocrine gut and pancreatic tumors. Acta Oncol 28: 425–431

    Article  Google Scholar 

  • O’Dorisio TM (1987) Sandostatin in the treatment of gastroenteropancreatic endocrine tumors. Springer, Berlin Heidelberg New York

    Google Scholar 

  • Parker CM, Hanke CW, Madura JA, Lisse EC (1984) Glucagonoma syndrome: case report and literature review. J Dermatol Surg Oncol 10: 884–889

    PubMed  CAS  Google Scholar 

  • Pauwels S, Leners N, Fiasse R, Jamar F (1994) Localization of gastroenteropancreatic neuroendocrine tumors with 111-Indium pentetreotide scintigraphy. Semin Oncol 21 [Suppl 13]: 15–20

    PubMed  CAS  Google Scholar 

  • Pisegna JR, Doppman JL, Norton JA, Metz DC, Jensen RT (1993) Prospective comparative study of ability of MR imaging and other imaging modalities to localize tumors in patients with Zollinger-Ellison syndrome. Dig Dis Sci 38: 1318–1328

    Article  PubMed  CAS  Google Scholar 

  • Prinz RA, Badrinath K, Banerji M, Sparagana M, Dorsch TR, Lawrence AM (1981) Operative and chemotherapeutic management of malignant glucagon producing tumors. Surgery 90: 713–719

    PubMed  CAS  Google Scholar 

  • Rich TA (1985) Radiation therapy for pancreatic cancer: eleven year experience at the JCRT. Int J Radiat Oncol Biol Phys 11: 759–763

    Article  PubMed  CAS  Google Scholar 

  • Rösch T, Lightdale CJ, Botet JF, Boyce GA, Sivak MV, Yasuda K, Heyder N, Palazzo L, Dancygier H, Schusdziarra V, Classen M (1992) Localization of pancreatic endocrine tumors by endoscopic ultrasonography. N Engl J Med 326: 1721–1726

    Article  PubMed  Google Scholar 

  • Rosenbaum A, Flourie B, Chagnon S, Blery M, Modigliani R (1989) Octreotide (SMS 201–995) in the treatment of metastatic glucagonoma: report of one case and review of the literature. Digestion 43: 116–120

    Article  Google Scholar 

  • Rossi P, Allison DJ, Bezzi M (1989) Radiology of the pancreas. Endocrine tumors of the pancreas. Radiol Clin North Am 27: 129–161

    PubMed  CAS  Google Scholar 

  • Ruszniewski P, Rougier P, Roche A, Legmann P, Sibert A, Hochlaf S, Ychou M, Mignon M (1993) Hepatic arterial chemoembolization in patients with liver metastases of endocrine tumors. A prospective phase II study in 24 patients. Cancer 71: 2624–2630

    Article  PubMed  CAS  Google Scholar 

  • Santangelo WC, Unger RH, Orci, L, Dueno MI, Popma JJ, Krejs GJ (1986) Somatostatin analog-induced remission of necrolytic migratory erythema without changes in plasma glucagon concentration. Pancreas 1: 464–469

    Article  PubMed  CAS  Google Scholar 

  • Sheehan-Dare RA, Simmons AV, Cotterill JA, Janke PG (1988) Hepatic tumors with hyperglucagonemia. Response to treatment with human lymphoblastoid interferon. Cancer 62: 912–914

    Article  PubMed  CAS  Google Scholar 

  • Shepherd ME, Raimer SS, Tyring SK, Smith EB (1991) Treatment of necrolytic migra- tory erythema in glucagonoma syndrome. J Am Acad Dermatol 25: 925–928

    Article  PubMed  CAS  Google Scholar 

  • Somers G, De Vroede M (1988) Islet cell tumors and diabetes mellitus. In: Lefèbvre PJ, Pipeleers DG (eds) The pathology of the endocrine pancreas in diabetes. Springer, Berlin Heidelberg New York, pp 171–190

    Google Scholar 

  • Stacpoole PW (1981) The glucagonoma syndrome: clinical features, diagnosis, and treatment. Endocr Rev 2: 347–361

    Article  PubMed  CAS  Google Scholar 

  • Thompson GB, van Heerden JA, Grant CS, Carney JA, Ilstrup DM (1988) Islet cell carcinomas of the pancreas: a twently-year experience. Surgery 104: 1011–1017

    PubMed  CAS  Google Scholar 

  • Torrisi JR, Treat J, Zeman R, Dritschilo A (1987) Radiotherapy in the management of pancreatic islet cell tumors. Cancer 60: 1226–1231

    Article  PubMed  CAS  Google Scholar 

  • van der Loos TLJM, Lambrecht ER, Lambers JCCA (1987) Successful treatment of glucagonoma-related necrolytic erythema with dacarbazine J Am Acad Dermatol 16: 468–472

    Google Scholar 

  • van Eyck CHJ, Bruining HA, Reubi J-C, Bakker WH, Oei HY, Krenning EP, Lamberts SWJ (1993) Use of isotope-labeled somatostatin analogs for visualization of islet cell tumors. World J Surg 17: 444–447

    Article  PubMed  Google Scholar 

  • Vinik AI, Moattari AR (1989) Treatment of endocrine tumors of the pancreas. Endocrinol Metab Clin North Am 18: 483–518

    PubMed  CAS  Google Scholar 

  • Wawrukiewicz AS, Rösch J, Keller FS, Lieberman DA (1982) Glucagonoma and its angiographic diagnosis. Cardiovasc Intervent Radiol 5: 318–324

    Article  PubMed  CAS  Google Scholar 

  • Wynick D, Anderson JV, Williams SJ, Bloom SR (1989) Resistance of metastatic pancreatic endocrine tumours after long-term treatment with the somatostatin analogue octreotide (SMS 201–995). Clin Endocrinol (Oxf) 30: 385–388

    Article  CAS  Google Scholar 

  • Wood SM, Polak JM, Bloom SR (1983) The glucagonoma syndrome. In: Lefèbvre PJ (ed) Glucagon II. Springer, Berlin Heidelberg New York, pp 411–430 (Handbook of experimental pharmacology, vol 66/11)

    Google Scholar 

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Scheen, A.J., Lefèbvre, P.J. (1996). Glucagonoma and Its Management. In: Lefèbvre, P.J. (eds) Glucagon III. Handbook of Experimental Pharmacology, vol 123. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-61150-6_15

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