Glucagonoma and Glucagonoma Syndrome: An Updated Review

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

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.

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.

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