Glucagonoma and pseudoglucagonoma syndrome.
BACKGROUND
Glucagonoma syndrome may present either associated with a pancreatic neoplasm which secretes glucagon or as a pseudo-glucagonoma associated with other diseases. It is extremely infrequent but well-known with a current prevalence estimated at 1/20,000,000.


METHODS
A retrospective review of glucagonoma and pseudoglucagonoma cases observed between January 1998 and December 2003 in three hospitals.


METHODS
Five cases: 3 with a demonstrable glucagon-secreting tumor and 2 cases without an associated neoplasm.


METHODS
Age, sex, initial diagnosis, associated symptoms, and pathology were analyzed as were procedures employed in diagnosis, imaging studies, laboratory data, surgery and follow-up.


RESULTS
Hyperglycemia and elevated plasma glucagon levels were found in all cases. In 3 cases, hypo-aminoacidemia and a descrease in fatty acids were found. No changes of zinc levels were observed. Abdominal ultrasound studies were of no value except in evaluating pancreatitis. A CT-scan was conclusive when a pancreatic neoplasm existed and 3 patients were operated on a curative basis.


CONCLUSIONS
Necrolytic migratory erythema was the key diagnosis in all cases. Surgery was intended to be curative. The follow-up was of 8, 37 and 57 months in the cases of true glucagonoma syndrome.


CONCLUSIONS
A real prevalence of glucagonoma syndrome could be greater than currently estimated. In our series, it was 13.5/20,000,000. Pseudoglucagonoma syndrome remains a rarity.
Echenique-Elizondo
Miguel
M
Department of Surgery, Basque Country University, San Sebastián, Spain. gepecelm@sc.ehu.es
Tuneu Valls
Ana
A
Elorza Orúe
José L
JL
Martinez de Lizarduy
Ignacio
I
Ibáñez Aguirre
Javier
J
D000368:Aged; D003937:Diagnosis, Differential; D005260:Female; D005934:Glucagon / Q000378:metabolism; D005935:Glucagonoma / Q000175:diagnosis* / Q000453:epidemiology / Q000378:metabolism; D006801:Humans; D008297:Male; D008875:Middle Aged; D010190:Pancreatic Neoplasms / Q000175:diagnosis* / Q000453:epidemiology / Q000378:metabolism; D012189:Retrospective Studies; D016896:Treatment Outcome
D005934:Glucagon