Glucagonoma and Glucagonoma Syndrome: An Updated Review

Abstract

Background: Glucagonoma is a rare well-differentiated slowly proliferating pancreatic neuroendocrine tumour, characterized by several manifestations including necrolytic migratory erythema, weight loss, diabetes and anaemia.

Aim: The purpose of the current review was to acknowledge literature about this rare tumour discerning the clinical features, diagnosis, treatment and prognosis of glucagonoma by comparing three different periods.

Results: A study published by Song reviewed 216 cases from studies published between 1999 and 2016, making a comparison with 407 cases reported before 1998 by Soga and Yakuwa. The current review consisted of 86 cases reported in studies published in and after 2017: 40 males and 46 females, with an average age of 48.2 years. The male-to-female ratio was 0.87. The rate of typical clinical findings was as follows: NME, 93.2% (69/74); DM, 70.3% (50/74); weight loss, 62.2% (46/74); anaemia, 44.6% (33/74); glossitis or stomatitis or cheilitis, 31.1% (23/74). A total of 85 cases reported the location of the tumour as the pancreas and 38.8% of these cases involved the tail of the pancreas. The average tumour size was 4.6 cm in patients between 2017 and 2024. Metastasis was detected in 52.3% of patients (45/86). The comparison with previous series highlighted an earlier age of diagnosis of glucagonoma and a higher rate of NME, consistent with a higher diagnostic accuracy.

Conclusions: Glucagonoma is a rare pathology, with peculiar characteristics that need to be acknowledged to achieve a timely diagnosis and finally improve patient prognosis.

Keywords: glucagonoma; glucagonoma syndrome; necrolytic migratory erythema.

Figure 1

Flow chart of the included studies.