II. Epidemiology

  1. Incidence: 1 to 32 cases per million person years

III. Pathophysiology

  1. Primary neuroendocrine tumor of the islet cell that secretes Insulin
    1. Associated with unregulated hyperinsulinemia due to excessive Insulin secretion from the islet cells
    2. Sporadic in most cases
      1. Associated with Multiple Endocrine Neoplasia Type 1 (MEN 1) in 6-7%
  2. Extrapancreatic tumors may also cause similar effects as a pareneoplastic syndrome
    1. Non-Islet cell tumors (NICTH) synthesize IGF-2 (or Insulin)
    2. Associated with Lung Cancer (including Mesothelioma), Sarcomas and gastrointestinal tumors

IV. Findings: Symptoms and Signs

V. Diagnosis: Lab findings consistent with Insulinoma

  1. Serum Glucose <55 mg/dl
  2. Insulin >=3 uU/ml
  3. C-Peptide >= 0.6 ng/ml
  4. Proinsulin >=5 pmol/L
  5. Beta Hydroxybutyrate <=2.7 mmol/L
  6. Negative Sulfonylurea screen

VI. Differential Diagnosis

VII. Imaging

  1. Localize tumor
    1. CT Abdomen
    2. MRI Abdomen
    3. Endoscopic Ultrasound
  2. Also consider the less common extrapancreatic source if islet tumor not identified
    1. Non-Islet cell tumors or NICTH (Paraneoplastic Syndrome cases

VIII. Management

  1. See Hypoglycemia
  2. Surgical excision
    1. Most primary islet cell tumors are benign and surgery is curative
  3. Medical Therapy (If surgery delayed or ineffective)
    1. Diazoxide 300-1200 mg/day IV/PO PLUS a Diuretic
    2. Octreotide 150-150 ug/day divided doses SC

IX. Resources

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