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Open Access Highly Accessed Case report

Glucagonoma syndrome: a case report

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 and Juan J González González

Author Affiliations

Department of General Surgery and Gastroenterology, Hospital Universitario Central de Asturias, Oviedo, Spain

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Journal of Medical Case Reports 2011, 5:402  doi:10.1186/1752-1947-5-402

Published: 22 August 2011

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.