A glucagonoma is a rare tumor of the alpha cells of the pancreas that results in up to a 1000-fold overproduction of the hormone glucagon. Alpha cell tumors are commonly associated with glucagonoma syndrome, though similar symptoms are present in cases of pseudoglucagonoma syndrome in the absence of a glucagon-secreting tumor. 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 International Statistical Classification of Diseases and Related Health Problems 10th Revision (ICD-10) is a coding of diseases and signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or diseases, as classified by the World Health Organization (WHO). ... // 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. ... For malignant tumors specifically, see cancer. ... Alpha cells are endocrine cells in the islets of Langerhans of the pancreas. ... The pancreas is a gland organ in the digestive and endocrine systems of vertebrates[2]. It is both exocrine (secreting pancreatic juice containing digestive enzymes) and endocrine (producing several important hormones, including insulin, glucagon, and somatostatin). ... Norepinephrine A hormone (from Greek όρμή - to set in motion) is a chemical messenger from one cell (or group of cells) to another. ... Glucagon ball and stick model A microscopic image stained for glucagon. ...

History

Fewer than 250 cases of glucagonoma have been described in the literature since their first description by Becker in 1942. Because of its rarity (fewer than one in 20 million worldwide), long-term survival rates are as yet unknown. Year 1942 (MCMXLII) was a common year starting on Thursday (the link will display the full 1942 calendar) of the Gregorian calendar. ...

Symptoms

The primary physiological effect of glucagonoma is an overproduction of the peptide hormone glucagon, which enhances blood glucose levels through the activation of catabolic processes including gluconeogenesis and lipolysis. Gluconeogenesis produces glucose from protein and amino acid materials; lipolysis is the breakdown of fat. The net result is hyperglucagonemia, decreased blood levels of amino acids (hypoaminoacidemia), anemia, diarrhea, and weight loss of 5-15 kg. This article or section does not cite any references or sources. ... Peptides (from the Greek πεπτος, digestible), are the family of short molecules formed from the linking, in a defined order, of various α-amino acids. ... Glucagon ball and stick model A microscopic image stained for glucagon. ... In medicine, blood sugar is glucose in the blood. ... Anabolism is the aspect of metabolism that contributes to growth. ... Pyruvic acid Oxaloacetic acid Phosphoenolpyruvate Fructose 1,6-bisphosphate Fructose 6-phosphate Glucose-6-phosphate Glucose Gluconeogenesis is the generation of glucose from non-sugar carbon substrates like pyruvate, lactate, glycerol, and amino acids (primarily alanine and glutamine). ... Lipolysis is the breakdown of fat stored in fat cells. ... Glucose (Glc), a monosaccharide (or simple sugar), is an important carbohydrate in biology. ... A representation of the 3D structure of myoglobin, showing coloured alpha helices. ... This article is about the class of chemicals. ... It has been suggested that Subcutaneous fat be merged into this article or section. ... This article is about the class of chemicals. ... This article discusses the medical condition. ... Types 5-7 on the Bristol Stool Chart are often associated with diarrhea Diarrhea (in American English) or diarrhoea (in British English) is a condition in which the sufferer has frequent watery, loose bowel movements (from the Greek word διάρροια; literally meaning through-flowing). Acute infectious diarrhea is a common cause... “Kg” redirects here. ...

Necrolytic migratory erythema (NME) is a classical symptom observed in patients with glucagonoma and is present in 80% of cases. Associated NME is characterized by the spread of erythematous blisters and swelling across areas subject to greater friction and pressure, including the lower abdomen, buttocks, perineum, and groin. Necrolytic migratory erythema (NME) is a classical symptom observed in patients with glucagonoma and is present in 80% of cases. ... For the human abdomen, see human abdomen. ... Bith buttocks. ... In human anatomy, the perineum, also called the taint, or gooch, is generally defined as the surface region in both males and females between the pubic symphysis and the coccyx. ... The groin is the crease at the junction of the torso with the legs and the adjacent region that includes the external genitals. ...

Diabetes mellitus also frequently results from the insulin and glucagon imbalance that occurs in glucagonoma.^[1] Diabetes mellitus is present in 80-90% of cases of glucagonoma, and is exacerbated by preexisting insulin resistance. For the disease characterized by excretion of large amounts of very dilute urine, see diabetes insipidus. ... is really just water but doctors get you to pay more Not to be confused with inulin. ... Glucagon ball and stick model A microscopic image stained for glucagon. ... Insulin resistance is the condition in which normal amounts of insulin are inadequate to produce a normal insulin response from fat, muscle and liver cells. ...

Diagnosis

A blood serum glucagon concentration of 1000 pg/mL or greater is indicative of glucagonoma (the normal range is 50-200 pg/mL). Blood plasma is a component of blood. ... The picogram is an SI unit of mass (symbol pg) defined as: 1 pg = 1 × 10-15 kilogram (1 × 10-12 gram) A picogram is one trillionth (1/1000000000000) of a gram. ... The millilitre (ml or mL, also spelt milliliter) is a metric unit of volume that is equal to one thousandth of a litre. ...

Blood tests may also reveal abnormally low concentrations of amino acids, zinc, and essential fatty acids, which are thought to play a role in the development of NME. Skin biopsies may also be taken to confirm the presence of NME. General Name, symbol, number zinc, Zn, 30 Chemical series transition metals Group, period, block 12, 4, d Appearance bluish pale gray Standard atomic weight 65. ... Essential fatty acids, or EFAs, are fatty acids that cannot be constructed within an organism from other components (generally all references are to humans) by any known chemical pathways; and therefore must be obtained from the diet. ... Brain biopsy A biopsy (in Greek: bios = life and opsy = look/appearance) is a medical test involving the removal of cells or tissues for examination. ...

A CBC can uncover anemia, which is an abnormally low level of hemoglobin. Schematics of shorthand for complete blood count commonly used by physicians. ... Structure of hemoglobin. ...

The tumor itself may be localized by any number of radiographic modalities, including angiography, CT, MRI, PET, and endoscopic ultrasound. Laparotomy is useful for obtaining histologic samples for analysis and confirmation of the glucagonoma. 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. ... negron305 Cat scan redirects here. ... “MRI” redirects here. ... Image of a typical positron emission tomography (PET) facility Positron emission tomography (PET) is a nuclear medicine medical imaging technique which produces a three-dimensional image or map of functional processes in the body. ... Endoscopic ultrasound is an ultrasound that is placed into the stomach and duodenum via the upper GI tract. ... A laparotomy is a surgical maneuver involving an incision through the abdominal wall to gain access into the abdominal cavity. ... A thin section of lung tissue stained with hematoxylin and eosin. ...

Treatment

Heightened glucagon secretion can be treated with the administration of octreotide, a somatostatin analog, which inhibits the release of glucagon.^[2] Doxorubicin and streptozotocin have also been used successfully to selectively damage alpha cells of the pancreatic islets. These do not destroy the tumor, but help to minimize progression of symptoms. Somatostatin is a hormone. ... Doxorubicin or Adriamycin® or hydroxyldaunorubicin is a DNA-interacting drug widely used in chemotherapy. ... Streptozotocin (STZ, Zanosar) is a drug used in treating cancer of the pancreas. ...

The only curative therapy for glucagonoma is surgical resection, where the tumor is removed. Resection has been known to reverse symptoms in some patients. “Surgeon” redirects here. ...

References

1. ^ Koike N, Hatori T, Imaizumi T, et al (2003). "Malignant glucagonoma of the pancreas diagnoses through anemia and diabetes mellitus". Journal of hepato-biliary-pancreatic surgery 10 (1): 101-5. PMID 12918465. 
2. ^ Moattari AR, Cho K, Vinik AI (1990). "Somatostatin analogue in treatment of coexisting glucagonoma and pancreatic pseudocyst: dissociation of responses". Surgery 108 (3): 581-7. PMID 2168587.