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Encyclopedia > Insulinoma
Insulinoma
Classification & external resources
Histopathology of pancreatic endocrine tumor (insulinoma).
ICD-10 C25.4, D13.7
ICD-9 157.4, 211.7
ICD-O: 8151
DiseasesDB 6830
eMedicine med/2677 
MeSH D007340

An insulinoma is a tumour of the pancreas derived from the beta cells which while retaining the ability to synthesize and secrete insulin is autonomous of the normal feedback mechanisms. Patients present with symptomatic hypoglycemia which is ameliorated by feeding. The diagnosis of an insulinoma is usually made biochemically with low blood sugar, elevated insulin, pro-insulin and C-peptide levels and confirmed by medical imaging or angiography. The definitive treatment is surgery. Image File history File linksMetadata No higher resolution available. ... The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The following codes are used with International Statistical Classification of Diseases and Related Health Problems. ... // C00-D48 - Neoplasms (C00-C14) Malignant neoplasms, lip, oral cavity and pharynx (C00) Malignant neoplasm of lip (C01) Malignant neoplasm of base of tongue (C02) Malignant neoplasm of other and unspecified parts of tongue (C03) Malignant neoplasm of gum (C04) Malignant neoplasm of floor of mouth (C05) Malignant neoplasm of... // C00-D48 - Neoplasms (C00-C14) Malignant neoplasms, lip, oral cavity and pharynx (C00) Malignant neoplasm of lip (C01) Malignant neoplasm of base of tongue (C02) Malignant neoplasm of other and unspecified parts of tongue (C03) Malignant neoplasm of gum (C04) Malignant neoplasm of floor of mouth (C05) Malignant neoplasm of... The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. ... The International Classification of Diseases for Oncology (ICD-O) is a domain specific extension of the International Statistical Classification of Diseases and Related Health Problems for tumor diseases. ... The Disease Bold textDatabase is a free website that provides information about the relationships between medical conditions, symptoms, and medications. ... eMedicine is an online clinical medical knowledge base that was founded in 1996. ... Medical Subject Headings (MeSH) is a huge controlled vocabulary (or metadata system) for the purpose of indexing journal articles and books in the life sciences. ... Tumor (American English) or tumour (British English) originally means swelling, and is sometimes still used with that meaning. ... The pancreas is an organ in the digestive and endocrine system (of vertebrates[2]). It is both exocrine (secreting pancreatic juice containing digestive enzymes) and endocrine (producing several important hormones, including insulin, glucagon, and somatostatin). ... Beta cells are a type of cell in the pancreas in areas called the islets of Langerhans. ... Insulin (from Latin insula, island, as it is produced in the Islets of Langerhans in the pancreas) is a polypeptide hormone that regulates carbohydrate metabolism. ... Hypoglycemia (hypoglycæmia in the UK) is a medical term referring to a pathologic state produced by a lower than normal amount of sugar (glucose) in the blood. ... Medical imaging designates the ensemble of techniques and processes used to create images of the human body (or parts thereof) for clinical purposes (medical procedures seeking to reveal, diagnose or examine disease) or medical science (including the study of normal anatomy and function). ...

Contents

Features

Insulinomas are rare neuroendocrine tumours with an incidence estimated at 1 to 4 new cases per million persons per year. Insulinoma is one of the most common types of tumour arising from the islets of Langerhans cells (pancreatic endocrine tumours). Estimates of malignancy (metastases) range from 5% to 30%. Over 99% of insulinomas originate in the pancreas, with rare cases from ectopic pancreatic tissue. About 5% of cases are associated with tumours of the parathyroid glands and the pituitary (Multiple endocrine neoplasia type 1) and are more likely to be multiple and malignant. Most insulinomas are small, less than 2 cm.
Neuroendocrine tumors, or more properly gastro-entero-pancreatic or gastroenteropancreatic neuroendocrine tumors (GEP-NETs), are cancers of the interface between the endocrine (hormonal) system and the nervous system. ... A porcine islet of Langerhans. ... Multiple endocrine neoplasia type 1 is part of a group of disorders that affect the endocrine system. ...


Signs and Symptoms

Patients with insulinomas usually develop neuroglycopenic symptoms. These include recurrent headache, lethargy, diplopia, and blurred vision, particularly with exercise or fasting. Severe hypoglycemia may result in seizures, coma, and permanent neurological damage. Symptoms resulting from the catecholaminergic response to hypoglycemia (i.e. tremulousness, palpitations, tachycardia, sweating, hunger, anxiety, nausea) are not as common. Sudden weight gain (the patient can become massively obese) is sometimes seen. Diplopia, commonly known as double vision, is the perception of two images from a single object. ... This article is about the medical term, epileptic seizure, as distinct from psychogenic non-epileptic seizure. ... In medicine, a coma (from the Greek koma, meaning deep sleep) is a profound state of unconsciousness. ... This article or section does not cite any references or sources. ...


Diagnosis

The diagnosis of insulinoma is suspected in a patient with symptomatic fasting hypoglycemia. The conditions of Whipple’s triad need to be met for the diagnosis of "true hypoglycemia" to be made:
Whipples triad or Whipples criteria is a medical term which refers to three conditions that are considered by physicians necessary for proving hypoglycemia as the cause of a persons symptoms. ...

1. symptoms and signs of hypoglycemia,
2. concomitant plasma glucose level of 45 mg/dL (2.5 mmol/L) or less, and
3. reversibility of symptoms with administration of glucose.

Blood tests

The following blood tests are needed to diagnose insulinoma:

  • glucose
  • insulin
  • C-peptide

If available, a proinsulin level might be useful as well. Other blood tests may help rule out other conditions which can cause hypoglycemia. Proinsulin is the substance made first in the pancreas that is then made into insulin. ...


Suppression tests

Normally, endogenous insulin production is suppressed in the setting of hypoglycemia. A 72-hour fast, usually supervised in a hospital setting, can be done to see if insulin levels fail to suppress, which is a strong indicator of the presence of an insulin-secreting tumour.

During the test, the patient may have calorie-free and caffeine-free liquids. Capillary blood glucose is measured every 4 hours using a reflectance meter, until values < 60 mg/dL (3.3 mmol/L) are obtained. Then, the frequency of blood glucose measurement is increased to every hour until values are < 49 mg/dL (2.7 mmol/L). At that point, or when the patient has symptoms of hypoglycemia, a blood test is drawn for serum glucose, insulin, proinsulin, and C-peptide levels. The fast is stopped at that point, and the hypoglycemia treated with intravenous dextrose or calorie-containing food or drink.

Four generations of blood glucose meter, c. ...

Diagnostic imaging

The insulinoma might be localized by non-invasive means, using ultrasound, CT scan, or by MRI techniques.
Sometimes, angiography with percutaneous transhepatic pancreatic vein catheterization to sample the blood for insulin levels is required. Calcium can be injected into selected arteries to stimulate insulin release from various parts of the pancreas, which can be measured by sampling blood from their respective veins. The use of calcium stimulation improves the specificity of this test.
During surgery to remove an insulinoma, an intra-operative ultrasound can sometimes localize the tumour, which helps guide the surgeon in the operation. Ultrasound is a form of cyclic sound pressure with a frequency greater than the upper limit of human hearing, this limit being approximately 20 kilohertz (20,000 hertz). ... CAT apparatus in a hospital Computed axial tomography (CAT), computer-assisted tomography, computed tomography, CT, or body section roentgenography is the process of using digital processing to generate a three-dimensional image of the internals of an object from a large series of two-dimensional X-ray images taken around... The mri are a fictional alien species in the Faded Sun Trilogy of C.J. Cherryh. ... Angiography or arteriography is a medical imaging technique in which an X-ray picture is taken to visualize the inner opening of blood filled structures, including arteries, veins and the heart chambers. ... The pancreatic veins consist of several small vessels which drain the body and tail of the pancreas, and open into the trunk of the lienal vein. ... Catheter disassembled In medicine, a catheter is a tube that a health professional may insert into part of the body. ... General Name, Symbol, Number calcium, Ca, 20 Chemical series alkaline earth metals Group, Period, Block 2, 4, s Appearance silvery white Standard atomic weight 40. ...


Treatment

The definitive management is surgical removal of the insulinoma. This may involve removing part of the pancreas as well (Whipple procedure and distal pancreatectomy).
Medications such as diazoxide and somatostatin can be used to block the release of insulin for patients who are not surgical candidates or who otherwise have inoperable tumours.
Streptozotocin is used in islet cell carcinomas which produce excessive insulin. Combination chemotherapy is used: either doxorubicin + streptozotocin, or fluorouracil + streptotozocin in patients where doxorubicin is contraindicated.[1]
In metastasizing tumours with intrahepatic growth, hepatic arterial occlusion or embolization can be used. [2] It has been suggested that this article or section be merged into Pancreaticoduodenectomy. ... Diazoxide is a potassium channel activator, which causes local relaxation in smooth muscle by increasing membrane permeability to potassium ions. ... Somatostatin is a hormone. ... Streptozotocin (STZ, Zanosar) is a drug used in treating cancer of the pancreas. ... Islet cell carcinoma is an uncommon cancer of the endocrine pancreas. ... Chemotherapy is the use of chemical substances to treat disease. ... Doxorubicin or Adriamycin® or hydroxyldaunorubicin is a DNA-interacting drug widely used in chemotherapy. ... Fluorouracil (5-FU) is a drug that is used in the treatment of cancer. ... Hepatic artery is an artery that distributes blood to the liver, pancreas and gallbladder as well as to the stomach and duodenal portion of the small intestine. ... A non-surgical, minimally-invasive procedure involving selective occlusion of blood vessels by purposefully introducing emboli to treat such conditions as aneurysms, epistaxis, and uterine fibroids. ...


Prognosis

Most patients with benign insulinomas can be cured with surgery. Persistent or recurrent hypoglycemia after surgery tends to occur in patients with multiple tumours. About two percent of patients develop diabetes mellitus after their surgery. For the disease characterized by excretion of large amounts of very dilute urine, see diabetes insipidus. ...


History

Hypoglycemia was first recognized in the 19th century. In the 1920’s, after the discovery of insulin and its use in the treatment of diabetics, hyperinsulinism was suspected to be a cause of hypoglycemia in non-diabetics. The first report of a surgical cure of hypoglycemia by removing an islet cell tumour was in 1929.


See also

Hypoglycemia (hypoglycæmia in the UK) is a medical term referring to a pathologic state produced by a lower than normal amount of sugar (glucose) in the blood. ... This list of causes of hypoglycemia is separated from the main article because of its length. ... The pancreas is an organ in the digestive and endocrine system (of vertebrates[2]). It is both exocrine (secreting pancreatic juice containing digestive enzymes) and endocrine (producing several important hormones, including insulin, glucagon, and somatostatin). ...

External links

References


  Results from FactBites:
 
Insulinoma - Wikipedia, the free encyclopedia (735 words)
An insulinoma is a tumour of the pancreas derived from the beta cells which while retaining the ability to synthesize and secrete insulin is autonomous of the normal feedback mechanisms.
The diagnosis of an insulinoma is usually made biochemically with low blood sugar, elevated insulin, pro-insulin and C-peptide levels and confirmed by medical imaging or angiography.
Insulinomas are rare neuroendocrine tumours with an incidence of 4 in 5 million.
  More results at FactBites »

 
 

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