Glucagonoma

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

  • Weight loss

  • Stomatitis

  • Thrombophlebitis

  • Anemia

  • Mild to moderate diabetes mellitus

Epidemiology

• • 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

Symptoms and Signs

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

  • Weight loss

  • Stomatitis

  • Thrombophlebitis

Laboratory Findings

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

  • Hypoaminoacidemia

  • Anemia

  • Hyperglycemia

Imaging Findings

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

• • 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

Rule Out

• • Other causes of dermatitis

  • Other pancreatic islet cell tumors

• • Elevated serum glucagon

  • Decreased serum amino acids

  • CT scan or MRI

When to Admit

• • Severe symptoms

• • Surgical resection of the primary tumor

  • -Location of tumor determines procedure

Surgery

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

  • Pancreaticoduodenectomy (for head)

  • Debulking of metastases

Indications

• • Whenever technically possible

  • Preoperative total parenteral nutrition should be administered for malnutrition

Medications

• • Total parenteral nutrition

  • Somatostatin for symptomatic palliation

  • Streptozocin and dacarbazine for unresectable lesions

Prognosis

• • Good with complete resection of the tumor

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

References