Skip to main content
Top
Published in: Journal of Medical Case Reports 1/2011

Open Access 01-12-2011 | Case report

Glucagonoma syndrome: a case report

Authors: Pablo Granero Castro, Alberto Miyar de León, Jose Granero Trancón, Paloma Álvarez Martínez, Jose A Álvarez Pérez, Jose C Fernández Fernández, Carmen M García Bernardo, Luis Barneo Serra, Juan J González González

Published in: Journal of Medical Case Reports | Issue 1/2011

Login to get access

Abstract

Introduction

Glucagonoma syndrome is a rare paraneoplastic phenomenon, with an estimated incidence of one in 20 million, characterized by necrolytic migratory erythema, hyperglucagonemia, diabetes mellitus, anemia, weight loss, glossitis, cheilitis, steatorrhea, diarrhea, venous thrombosis and neuropsychiatric disturbances in the setting of a glucagon-producing alpha-cell tumor of the pancreas. Necrolytic migratory erythema is the presenting manifestation in the majority of cases, so its early suspicion and correct diagnosis is a key factor in the management of the patient.

Case presentation

We present the case of a 70-year-old Caucasian woman with glucagonoma syndrome due to an alpha-cell tumor located in the tail of the pancreas, successfully treated with surgical resection.

Conclusion

Clinicians should be aware of the unusual initial manifestations of glucagonoma. Early diagnosis allows complete surgical resection of the neoplasm and provides the only chance of a cure.
Appendix
Available only for authorised users
Literature
1.
go back to reference Hellman P, Andersson M, Rastad J, Juhlin C, Karacagil S, Eriksson B, Skogseid B, Akerström G: Surgical strategy for large or malignant endocrine pancreatic tumors. World J Surg. 2000, 24: 1353-1360. 10.1007/s002680010224.CrossRefPubMed Hellman P, Andersson M, Rastad J, Juhlin C, Karacagil S, Eriksson B, Skogseid B, Akerström G: Surgical strategy for large or malignant endocrine pancreatic tumors. World J Surg. 2000, 24: 1353-1360. 10.1007/s002680010224.CrossRefPubMed
2.
go back to reference Okauchi Y, Nammo T, Iwahashi H, Kizu T, Hayashi I, Okita K, Yamagata K, Uno S, Katsube F, Matsuhisa M, Kato K, Aozasa K, Kim T, Osuga K, Nakamori S, Tamaki Y, Funahashi T, Miyagawa J, Shimomura I: Glucagonoma diagnosed by arterial stimulation and venous sampling (ASVS). Inter Med. 2009, 48: 1025-1030. 10.2169/internalmedicine.48.1676.CrossRef Okauchi Y, Nammo T, Iwahashi H, Kizu T, Hayashi I, Okita K, Yamagata K, Uno S, Katsube F, Matsuhisa M, Kato K, Aozasa K, Kim T, Osuga K, Nakamori S, Tamaki Y, Funahashi T, Miyagawa J, Shimomura I: Glucagonoma diagnosed by arterial stimulation and venous sampling (ASVS). Inter Med. 2009, 48: 1025-1030. 10.2169/internalmedicine.48.1676.CrossRef
3.
go back to reference Wermers RA, Fatourechi V, Wynne AG, Kvols LK, Lloyd RV: The glucagonoma syndrome. Clinical and pathologic features in 21 patients. Medicine. 1996, 75: 53-63. 10.1097/00005792-199603000-00002.CrossRefPubMed Wermers RA, Fatourechi V, Wynne AG, Kvols LK, Lloyd RV: The glucagonoma syndrome. Clinical and pathologic features in 21 patients. Medicine. 1996, 75: 53-63. 10.1097/00005792-199603000-00002.CrossRefPubMed
4.
go back to reference Chastain MA: The glucagonoma syndrome: a review of its features and discussion of new perspectives. Am J Med Sci. 2001, 321: 306-320. 10.1097/00000441-200105000-00003.CrossRefPubMed Chastain MA: The glucagonoma syndrome: a review of its features and discussion of new perspectives. Am J Med Sci. 2001, 321: 306-320. 10.1097/00000441-200105000-00003.CrossRefPubMed
5.
go back to reference Stacpoole PW: The glucagonoma syndrome: clinical features, diagnosis, and treatment. Endocr Rev. 1981, 2: 347-361. 10.1210/edrv-2-3-347.CrossRefPubMed Stacpoole PW: The glucagonoma syndrome: clinical features, diagnosis, and treatment. Endocr Rev. 1981, 2: 347-361. 10.1210/edrv-2-3-347.CrossRefPubMed
6.
7.
go back to reference van Beek AP, de Haas ER, van Vloten WA, Lips CJ, Roijers JF, Canninga-van Dijk MR: The glucagonoma syndrome and necrolytic migratory erythema: a clinical review. Eur J Endocrinol. 2004, 151: 531-537. 10.1530/eje.0.1510531.CrossRefPubMed van Beek AP, de Haas ER, van Vloten WA, Lips CJ, Roijers JF, Canninga-van Dijk MR: The glucagonoma syndrome and necrolytic migratory erythema: a clinical review. Eur J Endocrinol. 2004, 151: 531-537. 10.1530/eje.0.1510531.CrossRefPubMed
8.
go back to reference Lobo I, Carvalho A, Amaral C, Machado S, Carvalho R: Glucagonoma syndrome and necrolytic migratory erythema. Int J Dermatol. 2010, 49: 24-29. 10.1111/j.1365-4632.2009.04220.x.CrossRefPubMed Lobo I, Carvalho A, Amaral C, Machado S, Carvalho R: Glucagonoma syndrome and necrolytic migratory erythema. Int J Dermatol. 2010, 49: 24-29. 10.1111/j.1365-4632.2009.04220.x.CrossRefPubMed
9.
go back to reference Teixeira RC, Nico MM, Ghideti AC: Necrolytic migratory erythema associated with glucagonoma: a report of 2 cases. Clinics (Sao Paulo). 2008, 63: 267-270.CrossRef Teixeira RC, Nico MM, Ghideti AC: Necrolytic migratory erythema associated with glucagonoma: a report of 2 cases. Clinics (Sao Paulo). 2008, 63: 267-270.CrossRef
10.
go back to reference Norton JA, Kivlen M, Li M, Schneider D, Chuter T, Jensen RT: Morbidity and mortality of aggressive resection in patients with advanced neuroendocrine tumors. Arch Surg. 2003, 138: 859-866. 10.1001/archsurg.138.8.859.CrossRefPubMed Norton JA, Kivlen M, Li M, Schneider D, Chuter T, Jensen RT: Morbidity and mortality of aggressive resection in patients with advanced neuroendocrine tumors. Arch Surg. 2003, 138: 859-866. 10.1001/archsurg.138.8.859.CrossRefPubMed
11.
go back to reference Öberg K: Pancreatic endocrine tumors. Semin Oncol. 2010, 37: 594-618. 10.1053/j.seminoncol.2010.10.014.CrossRefPubMed Öberg K: Pancreatic endocrine tumors. Semin Oncol. 2010, 37: 594-618. 10.1053/j.seminoncol.2010.10.014.CrossRefPubMed
12.
go back to reference Papavramidis T, Papavramidis S: Solid pseudopapillary tumors of the pancreas: review of 718 patients reported in English literature. J Am Coll Surg. 2005, 100: 965-972.CrossRef Papavramidis T, Papavramidis S: Solid pseudopapillary tumors of the pancreas: review of 718 patients reported in English literature. J Am Coll Surg. 2005, 100: 965-972.CrossRef
13.
go back to reference Konukiewitz B, Enosawa T, Klöppel G: Glucagon expression in cystic pancreatic neuroendocrine neoplasms: an immunohistochemical analysis. Virchows Arch. 2011, 458: 47-53. 10.1007/s00428-010-0985-6.CrossRefPubMed Konukiewitz B, Enosawa T, Klöppel G: Glucagon expression in cystic pancreatic neuroendocrine neoplasms: an immunohistochemical analysis. Virchows Arch. 2011, 458: 47-53. 10.1007/s00428-010-0985-6.CrossRefPubMed
14.
go back to reference Schwartz RA: Glucagonoma and pseudoglucagonoma syndromes. Int J Dermatol. 1997, 36: 81-89. 10.1046/j.1365-4362.1997.00042.x.CrossRefPubMed Schwartz RA: Glucagonoma and pseudoglucagonoma syndromes. Int J Dermatol. 1997, 36: 81-89. 10.1046/j.1365-4362.1997.00042.x.CrossRefPubMed
Metadata
Title
Glucagonoma syndrome: a case report
Authors
Pablo Granero Castro
Alberto Miyar de León
Jose Granero Trancón
Paloma Álvarez Martínez
Jose A Álvarez Pérez
Jose C Fernández Fernández
Carmen M García Bernardo
Luis Barneo Serra
Juan J González González
Publication date
01-12-2011
Publisher
BioMed Central
Published in
Journal of Medical Case Reports / Issue 1/2011
Electronic ISSN: 1752-1947
DOI
https://doi.org/10.1186/1752-1947-5-402

Other articles of this Issue 1/2011

Journal of Medical Case Reports 1/2011 Go to the issue