CASE REPORT   

Gazzetta Medica Italiana - Archivio per le Scienze Mediche 2021 November;180(11):764-7

DOI: 10.23736/S0393-3660.19.04280-3

Copyright © 2019 EDIZIONI MINERVA MEDICA

lingua: Inglese

Death as a consequence of a rare disease: glucagonoma

Luigi CASTORANI ✉, Giuseppe DIPAOLA

Department of Emergency Medicine, MeCAU Hospital, ASL BT, Barletta, Barletta-Andria-Trani, Italy

Glucagonoma is a rare neuroendocrine tumor of the pancreas. It is characterized by some symptoms: diabetes mellitus, migrating necrolytic erythema (it is considered to be the hallmark of glucagonoma syndrome and is characterized by an annular pattern of erythema with centrally formed fragile vesicles, bullae and crusts) anemia, weight loss, venous thrombosis, psychiatric symptoms (depression). The incidence in the general population is 1: 20,000,000. Glucagonoma usually occurs during the fifth decade of life. It causes the production of high glucagon levels by islet α-cells that contributes to the paraneoplastic phenomenon, namely glucagonoma syndrome. Etiology is unknown. Most glucagonomas already have metastases before diagnosis, especially to the liver. The median time between onset of glucagonoma syndrome and diagnosis is 3-4 years. Diagnosis is based on clinical signs and elevated glucagon values. The treatment is both pharmacological (with the administration of somatostatin analogues as octreotide or lanreotide, administration of insulin, chemotherapy) and surgical (synchronous resection of liver metastasis or liver transplantation). Metastasis represents the main prognostic factor for glucagonoma. The glucagonomic prognosis is usually unfavorable, as most of these tumors have already metastasized before the clinical diagnosis. The main problem is the diagnostic delay, which causes serious complications when diagnosed. The objective of this case report was to show how the diagnostic delay determined the patient’s death and to focus on the characteristic symptoms of this neoplasm.

KEY WORDS: Neuroendocrine tumors; Glucagonoma; Necrolytic migratory erythema