Glucagonoma syndrome: a case report

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.

Figure 1

Necrolytic migratory erythema. (A) Skin lesions affecting pretibial area. (B) Skin biopsy in necrolytic migratory erythema showing a zone of necrolysis and vacuolated keratinocytes.

Figure 2

Radiological and histological findings. (A) Axial and (B) coronal CT scan revealing a 5-7 cm nodular mass in the tail of her pancreas (*)(C) Histological examination of the mass showing an alpha-cell pancreatic tumor (hematoxylin and eosin ×20). (D) Inmunostaining revealed numerous glucagon-positive cells (×20).