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The glucagonoma syndrome and its management
Journal of Surgical Oncology
|January 1, 1977
Summary
Glucagonoma syndrome, linked to islet cell tumors and hyperglucagonemia, presents with diverse symptoms like anemia and skin rash. Surgical removal of the tumor can reverse the condition.
Area of Science:
- Endocrinology
- Oncology
Background:
- Glucagonoma syndrome is associated with islet cell tumors causing hyperglucagonemia.
- It affects some patients, presenting with a range of clinical manifestations.
Observation:
- Key symptoms include anemia, diabetes mellitus, characteristic skin rash, glossitis, stomatitis, weight loss, diarrhea, and venous thromboses.
- Affected individuals often exhibit low plasma amino acid levels and jejunal/ileal mucosal changes.
- While most patients are postmenopausal women, affected individuals range from 40 to 65 years old.
Findings:
- Hyperglucagonemia stems from elevated proglucagon and true glucagon immunoreactive fractions.
- Twenty-two probable cases have been reported, with twelve confirmed by glucagon levels.
Implications:
- Surgical resection of the tumor offers a potential cure for glucagonoma syndrome.
- Metastatic disease management involves chemotherapy, with streptozotocin and DTIC showing some response.
- Rash management may involve antibiotics or corticosteroids, though often with limited success.
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