Abstract
Glucagonoma is a rare tumor of pancreatic islet cells. The hyperglucagonemia causes a paraneoplastic syndrome, of which the most well-known feature is a migratory rash, called necrolytic migratory erythema. Other findings include diabetes, weight loss, anemia, thromboembolic disease, and depression. Diagnosis is often delayed, and many patients present with advanced or metastatic disease. Successful treatment leads to rapid resolution of symptoms, and surgery should be considered even in patients who cannot be rendered disease free, in order to achieve symptom reduction.
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References
Becker SW, Kahn, D, Rothman, S. Cutaneous manifestations of internal malignant tumours. Arch Dermatol Syph. 1942;49:1069.
Goessner W, Korting GW. Metastasizing islet cell carcinoma of the A cell type in a case of pemphigus foliaceus with diabetes renalis. Dtsch Med Wochenschr. 1960;85:434–7.
McGavran MH, Unger RH, Recant L, Polk HC, Kilo C, Levin ME. A glucagon-secreting alpha-cell carcinoma of the pancreas. N Engl J Med. 1966;274(25):1408–13.
Zhdanov VS. Diabetes in tumor of islands of Langerhans. Arkh Patol. 1956;18(3):92–3.
Friesen SR, editor. Surgical endocrinology: clinical syndromes. Philadelphia: J. B. Lippincott Company; 1978:120–144.
Wilkinson GT, Prydie J, Scarnell J. Possible “orf” (contagious pustular dermatitis, contagious ecthyma of sheep) infection in the dog. Vet Rec. 1970;87(25):766–7.
Mallinson CN, Bloom SR, Warin AP, Salmon PR, Cox B. A glucagonoma syndrome. Lancet. 1974;2(7871):1–5.
Wermers RA, Fatourechi V, Wynne AG, Kvols LK, Lloyd RV. The glucagonoma syndrome. Clinical and pathologic features in 21 patients. Medicine. 1996;75(2):53–63.
Frankton S, Bloom SR. Gastrointestinal endocrine tumours. Glucagonomas. Baillieres Clin Gastroenterol. 1996;10(4):697–705.
Wilkinson DS. Necrolytic migratory erythema with carcinoma of the pancreas. Trans St Johns Hosp Dermatol Soc. 1973;59(2):244–50.
Chastain MA. The glucagonoma syndrome: a review of its features and discussion of new perspectives. Am J Med Sci. 2001;321(5):306–20.
Prinz RA, Dorsch TR, Lawrence AM. Clinical aspects of glucagon-producing islet cell tumors. Am J Gastroenterol. 1981;76(2):125–31.
Shupack JL, Berczeller PH, Stevens DM. The glucagonoma syndrome. J Dermatol Surg Oncol. 1978;4(3):242–7.
Kheir SM, Omura EF, Grizzle WE, Herrera GA, Lee I. Histologic variation in the skin lesions of the glucagonoma syndrome. Am J Surg Pathol. 1986;10(7):445–53.
Wilkinson DS. Necrolytic migratory erythema with pancreatic carcinoma. Proc R Soc Med. 1971;64:1196.
Necrolytic migratory erythema with carcinoma of the pancrease. Trans St Johns Hosp Dermatol Soc. 1973;59:244.
Sweet RD. A dermatosis specifically associated with a tumour of pancreatic alpha cells. Br J Dermatol. 1974;90(3):301–8.
Pedersen NB, Jonsson L, Holst JJ. Necrolytic migratory erythema and glucagon cell tumour of the pancreas: the glucagonoma syndrome. Report of two cases. Acta Derm Venereol. 1976;56(5):391–5.
Boden G. Insulinoma and glucagonoma. Semin Oncol. 1987;14(3):253–62.
Mozell E, Stenzel P, Woltering EA, Rosch J, O’Dorisio TM. Functional endocrine tumors of the pancreas: clinical presentation, diagnosis, and treatment. Curr Probl Surg. 1990;27(6):301–86.
Stacpoole PW. The glucagonoma syndrome: clinical features, diagnosis, and treatment. Endocr Rev. 1981;2(3):347–61.
Kaplan EL, Michelassi F. Endocrine tumors of the pancreas and their clinical syndromes. Surg Annu. 1986;18:181–223.
Khandekar JD, Sriratana P. Response of glucagonoma syndrome to lomustine. Cancer Treat Rep. 1986;70(3):433–4.
Lambrecht ER, van der Loos TL, van der Eerden AH. Retrobulbar neuritis as the first sign of the glucagonoma syndrome. Int Ophthalmol. 1987;11(1):13–5.
Croughs RJ, Hulsmans HA, Israel DE, Hackeng WH, Schopman W. Glucagonoma as part of the polyglandular adenoma syndrome. Am J Med. 1972;52(5):690–8.
Lefebvre PJ. Glucagon and its family revisited. Diabetes Care. 1995;18(5):715–30.
Parker CM, Hanke CW, Madura JA, Liss EC. Glucagonoma syndrome: case report and literature review. J Dermatol Surg Oncol. 1984;10(11):884–9.
Schwartz RA. Glucagonoma and pseudoglucagonoma syndromes. Int J Dermatol. 1997;36(2):81–9.
Mayo SC, de Jong MC, Pulitano C, Clary BM, Reddy SK, Gamblin TC, et al. Surgical management of hepatic neuroendocrine tumor metastasis: results from an international multi-institutional analysis. Ann Surg Oncol. 2010;17(12):3129–36.
Smith AP, Doolas A, Staren ED. Rapid resolution of necrolytic migratory erythema after glucagonoma resection. J Surg Oncol. 1996;61(4):306–9.
Edney JA, Hofmann S, Thompson JS, Kessinger A. Glucagonoma syndrome is an underdiagnosed clinical entity. Am J Surg. 1990;160(6):625–8; discussion 8–9.
Holmes A, Kilpatrick C, Proietto J, Green MD. Reversal of a neurologic paraneoplastic syndrome with octreotide (Sandostatin) in a patient with glucagonoma. Am J Med. 1991;91(4):434–6.
Mazzaglia PJ, Berber E, Milas M, Siperstein AE. Laparoscopic radiofrequency ablation of neuroendocrine liver metastases: a 10-year experience evaluating predictors of survival. Surgery. 2007;142(1):10–9.
Gupta S, Johnson MM, Murthy R, Ahrar K, Wallace MJ, Madoff DC, et al. Hepatic arterial embolization and chemoembolization for the treatment of patients with metastatic neuroendocrine tumors: variables affecting response rates and survival. Cancer. 2005;104(8):1590–602.
Saxena A, Chua TC, Bester L, Kokandi A, Morris DL. Factors predicting response and survival after Yttrium-90 radioembolization of unresectable neuroendocrine tumor liver metastases: a critical appraisal of 48 cases. Ann Surg. 2010;251(5):910–6.
Kennedy AS, Dezarn WA, McNeillie P, Coldwell D, Nutting C, Carter D, et al. Radioembolization for unresectable neuroendocrine hepatic metastases using resin 90Y-microspheres: early results in 148 patients. Am J Clin Oncol. 2008;31(3):271–9.
Broder LE, Carter SK. Pancreatic islet cell carcinoma. I. Clinical features of 52 patients. Ann Intern Med. 1973;79(1):101–7.
Wynick D, Hammond PJ, Bloom SR. The glucagonoma syndrome. Clin Dermatol. 1993;11(1):93–7.
Arnold JC, O’Grady JG, Bird GL, Calne RY, Williams R. Liver transplantation for primary and secondary hepatic apudomas. Br J Surg. 1989;76(3):248–9.
Jockenhovel F, Lederbogen S, Olbricht T, Schmidt-Gayk H, Krenning EP, Lamberts SW, et al. The long-acting somatostatin analogue octreotide alleviates symptoms by reducing posttranslational conversion of prepro-glucagon to glucagon in a patient with malignant glucagonoma, but does not prevent tumor growth. Clin Investig. 1994;72(2):127–33.
Yao JC, Shah MH, Ito T, Bohas CL, Wolin EM, Van Cutsem E, et al. Everolimus for advanced pancreatic neuroendocrine tumors. N Engl J Med. 2011;364(6):514–23.
Raymond E, Dahan L, Raoul JL, Bang YJ, Borbath I, Lombard-Bohas C, et al. Sunitinib malate for the treatment of pancreatic neuroendocrine tumors. N Engl J Med. 2011;364(6):501–13.
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Mazzaglia, P. (2015). Glucagonoma. In: Pasieka, J., Lee, J. (eds) Surgical Endocrinopathies. Springer, Cham. https://doi.org/10.1007/978-3-319-13662-2_52
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DOI: https://doi.org/10.1007/978-3-319-13662-2_52
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