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  • • Migratory necrolytic dermatitis, usually involving the legs and perineum

    • Weight loss

    • Stomatitis

    • Thrombophlebitis

    • Anemia

    • Mild to moderate diabetes mellitus

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Epidemiology

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  • • Arise from cells in the pancreatic islets

    • Most tumors are solitary and large (> 4 cm) located in the body or tail of the pancreas

    • About 25% are benign and confined to the pancreas

    • -The remainder has metastasized by the time of diagnosis, most often to the liver, lymph nodes, adrenal gland, or vertebrae

    • The age range is 20-70 years, and the condition is more common in women

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Symptoms and Signs

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  • • Migratory necrolytic dermatitis, usually involving the legs and perineum

    • Weight loss

    • Stomatitis

    • Thrombophlebitis

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Laboratory Findings

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  • • Elevated serum glucagon level (> 1000 pg/mL)

    • Hypoaminoacidemia

    • Anemia

    • Hyperglycemia

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Imaging Findings

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  • • CT scan or MRI demonstrates the tumor and sites of metastases

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  • • The diagnosis may be suspected from the distinctive skin lesion

    • -In fact, the presence of a prominent rash in a patient with diabetes mellitus should be enough to raise suspicions

    • Glucagonoma should also be suspected in any patient with new onset of diabetes after age 60

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Rule Out

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  • • Other causes of dermatitis

    • Other pancreatic islet cell tumors

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  • • Elevated serum glucagon

    • Decreased serum amino acids

    • CT scan or MRI

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When to Admit

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  • • Severe symptoms

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  • • Surgical resection of the primary tumor

    • -Location of tumor determines procedure

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Surgery

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  • • Distal subtotal pancreatectomy and splenectomy (for head and body)

    • Pancreaticoduodenectomy (for head)

    • Debulking of metastases

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Indications

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  • • Whenever technically possible

    • Preoperative total parenteral nutrition should be administered for malnutrition

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Medications

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  • • Total parenteral nutrition

    • Somatostatin for symptomatic palliation

    • Streptozocin and dacarbazine for unresectable lesions

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Prognosis

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  • • Good with complete resection of the tumor

    • Palliation can be achieved with resection and debulking of metastatic disease

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References

Chastain MA. The glucagonoma syndrome: a review of its features and discussion of new perspectives. Am J Med Sci. 2001;321:306.  [PubMed: 11370794]

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