The glucagonoma syndrome and its management

Anne Kessinger, Henry M. Lemon, John F. Foley

Research output: Contribution to journalArticle

36 Scopus citations

Abstract

The glucagonoma syndrome occurs in some but not all patients with a benign or malignant islet cell tumor and hyperglucagonemia. Manifestations may include anemia, diabetes mellitus, pruritic skin rash, glossitis, stomatitis, weight loss, diarrhea, flexible fingernails, venous thromboses, low plasma amino acid levels, and coarse folds of the jejunum and ileum. Most patients are postmenopausal women, but men and women ages 40 to 65 have been affected. The course is variable depending upon the nature of the underlying tumor. Twenty‐two cases of probable glucagonoma syndrome have been reported; twelve documented with glucagon levels. The hyperglucagonemia results from elevation of the proglucagon and true glucagon immunoreactive fractions of pancreatic glucagon. Management of the rash can be accomplished rarely with topical or systemic antibiotics or corticosteroids. If the tumor is resectable, surgery reverses the syndrome. Patients with metastatic disease have responded to streptozotocin and DTIC.

Original languageEnglish (US)
Pages (from-to)419-424
Number of pages6
JournalJournal of Surgical Oncology
Volume9
Issue number5
DOIs
Publication statusPublished - 1977

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Keywords

  • glucagonoma syndrome
  • islet cell carcinoma

ASJC Scopus subject areas

  • Surgery
  • Oncology

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