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Glucagonoma syndrome.

S R Bloom, J M Polak

    The American Journal of Medicine
    |May 29, 1987
    PubMed
    Summary
    This summary is machine-generated.

    Glucagonoma syndrome, marked by a distinctive rash and diabetes, is diagnosed by high plasma glucagon. Treatment includes surgery, embolization, chemotherapy, or somatostatin analogues for symptom management.

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    Area of Science:

    • Endocrinology
    • Oncology

    Background:

    • Glucagonoma syndrome presents with a characteristic necrolytic migratory rash, diabetes mellitus, weight loss, and thrombosis.
    • Diagnosis relies on elevated plasma glucagon levels, excluding other causes like renal failure or stress.
    • Pancreatic alpha-cell tumors secreting glucagon are the underlying cause, identifiable via immunocytochemistry.

    Purpose of the Study:

    • To outline the diagnostic criteria for glucagonoma syndrome.
    • To review current and novel therapeutic strategies for managing glucagonomas and their associated symptoms.
    • To explore the role of somatostatin analogues in treating glucagonoma.

    Main Methods:

    • Diagnostic confirmation through measurement of plasma glucagon concentrations.
    • Identification of pancreatic alpha-cell tumors using immunocytochemistry.

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  • Evaluation of treatment outcomes including surgery, hepatic artery embolization, chemotherapy (5-fluorouracil and streptozotocin), and somatostatin analogue SMS 201-995.

    • Main Results:

    • Surgical removal is optimal but often hindered by metastasis at diagnosis (approx. 50%).
    • Hepatic artery embolization can induce remission in hepatic secondaries.
    • Somatostatin analogue SMS 201-995 effectively suppresses glucagon secretion and can lead to clinical remission in some patients.

    Conclusions:

    • Glucagonoma syndrome requires prompt diagnosis and a multimodal treatment approach.
    • While surgery is primary, palliative treatments like embolization, chemotherapy, and somatostatin analogues offer management options for metastatic disease.
    • Somatostatin analogues represent a promising therapeutic advancement for managing glucagonoma symptoms and disease progression.