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Diabetes Information                                        

Insulinomas


Alternative names

Insuloma; Islet cells adenoma

Definition   

An insulinoma is a tumor in the pancreas that produces too much insulin.

Causes, incidence, and risk factors 

The pancreas is an organ in the abdomen that makes the hormone insulin. Insulin is required to regulate blood sugar levels. Tumors of the pancreas that produce too much insulin (hyperinsulinemia) are called insulinomas.

High insulin levels cause low blood glucose (sugar), also called hypoglycemia. The hypoglycemia may be mild, leading to symptoms such as anxiety and hunger, or severe, leading to seizures, coma, and even death.

Insulinomas are rare tumors. They usually occur as single, small tumors in adults. They are very rare in children. Most children with hyperinsulinism have multiple pockets of overactive insulin-secreting cells in the pancreas, rather than a single discrete tumor.

Most insulinomas are not cancer. However, about five to ten percent are cancer. People with the genetic syndrome called multiple endocrine neoplasia Type I (MENI) are at risk for developing insulinomas.

Symptoms    Return to top

Signs and tests    Return to top

The person's blood may be tested while fasting (not eating). The person may have:

  • low blood glucose
  • high serum insulin level
  • high C-peptide level

Other tests may include:

  • CT scan or MRI of the abdomen to look for a pancreatic tumor
  • Endoscopic ultrasound to look for a pancreatic tumor (when CT or MRI scan is normal)
  • Octreotide scan to look for a pancreatic tumor (when CT or MRI scan is normal)
  • Pancreatic arteriography (when CT or MRI scan is normal)
  • Pancreatic venous sampling for insulin (when CT or MRI scan is normal)

Treatment    Return to top

Surgery is the preferred treatment for insulinoma. The location of the tumor is determined using diagnostic testing or surgical exploration. Single tumors are removed, but patients with multiple tumors usually require partial removal of the pancreas (partial pancreatectomy). At least 15% of the pancreas is left to prevent nutrient malabsorption from lack of pancreatic enzymes.

If no tumor is found during surgery or a patient is not a candidate for surgery, the drug diazoxide may be given to lower insulin secretion and avoid hypoglycemia. A diuretic (water pill) is given with this medication to keep the person's body from retaining fluid.

Octreotide has been used to suppress insulin secretion in some patients. Medication is also used to stabilize the person prior to surgery.

Expectations (prognosis)    Return to top

In a majority of cases, the tumor is benign and surgery is effective. However, a severe hypoglycemic reaction or the spread of cancerous tumors to other organs can be fatal.

Complications    Return to top

  • severe hypoglycemic reaction
  • cancerous tumor spreading (metastasis)

Calling your health care provider    Return to top

Call your health care provider if symptoms of insulinoma develop. Convulsions and decreased consciousness are emergency symptoms.

 

 

Insulinoma (beta cell tumor)

top

Only 7-10% behave malignantly (perhaps because most are detected when very small due to symptoms)

Functional status is NOT an independent prognostic factor

Associated with hyperinsulinemia; usually adults; <10% associated with MEN1 syndrome

Case report of widely metastatic tumor with focal rhabdomyosarcomatous areas, AJSP 1989;13:422

Whipple’s triad: symptoms of hypoglycemia (stupor, confusion, loss of consciousness), glucose < 50 mg/dl, symptoms relieved by glucose or symptoms caused by fasting or exercise

Laboratory: high insulin levels and high insulin/glucose ratio; hypoglycemia is mild in 80% of cases

Diagnosis: high insulin levels, intravenous tolbutamide administration detects serum proinsulin

Use arteriography (60% successful) or ultrasound to locate small tumors

Treatment: surgical exploration or subtotal pancreatectomy

Benign (90%): solitary, encapsulated, 1.5 cm or less; solid/gyriform, no glands

Malignant (10%): based on local invasion or metastases; usually causes more pronounced hypoglycemia

Note: amyloid may be derived from somatostatin and not amylin, AJSP 1998;22:360

Micro: solid or gyriform patterns, usually without glands; in children are associated with nesidioblastosis (direct transformation of ductal epithelium into neoplastic islet tissue); amyloid present, Archives 1978;102:227

Positive stains: insulin (less than normal beta cells), proinsulin (50%), chromogranin, amylin

Micro images: insulin immunostain#1, #2

EM: round secretory granules with irregular crystals separated from enclosing membrane by a distinct halo; granules do NOT imply functional activity

DD of hyperinsulinism: diffuse hyperplasia of the islets in infants of diabetic mothers

DD of hypoglycemia: insulin sensitivity, diffuse liver disease, glycogenoses, solitary fibrous tumor of pleura / peritoneum (tumor cells secrete insulin like growth factor II), hepatocellular carcinomas

 

 

 

 

         

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Page Updated 12/14/2005