Gucagonoma syndrome is an underdiagnosed clinical entity

James A. Edney, Scott Hofmann, Jon S. Thompson, Anne Kessinger

Research output: Contribution to journalArticle

42 Scopus citations

Abstract

Glucagonomas, considered among the rarest of the islet cell neoplasms, produce a well-defined clinical syndrome characterized by necrolytic migratory erythema, diabetes mellitus, glossitis, anemia, and weight loss. This report describes seven patients with glucagonoma treated at our institution. All seven had the characteristic dermatologic manifestations, present from 1 to 6 years prior to diagnosis. Five patients had extensive disease at the time of initial operation, three of whom underwent aggressive cytoreductive surgery, whereas the other two had biopsy only. The remaining two patients presented with a single nodule each, underwent distal pancreatectomy and splenectomy, and remain free of disease 2 and 6 years postoperatively. Earlier recognition of the distinctive physical findings peculiar to this syndrome should increase survival. Aggressive cytoreductive surgery results in prolonged remission.

Original languageEnglish (US)
Pages (from-to)625-629
Number of pages5
JournalThe American Journal of Surgery
Volume160
Issue number6
DOIs
StatePublished - Dec 1990

ASJC Scopus subject areas

  • Surgery

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