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Glucagonoma

Glucagonoma — a rare insular and cellular tumor a lot of producing a glucagon (the tab. a glucagonoma).

Etiology

The glucagonoma in 70% of cases of a zlokachestvenn (in 80% for the moment of diagnosis metastasises are defined), is most often localized in the tail of a pancreas and at the time of diagnosis, as a rule, exceeds in the diameter of 5 cm (does not represent problems with topical diagnosis). Multicentric tumors are found in 10-12% of cases.

Pathogeny

Hyperproduction of a glucagon and tumoral progression is its cornerstone. A lot of glucagon disturbances of carbohydrate metabolism (strengthening of a hepatic gluconeogenesis and glycolysis), and are connected with disturbance of an exchange of amino acids — changes from skin. Meets within a syndrome of multiple endocrine neoplaziya of 1 type.

Epidemiology

Meets casuistic seldom.

Clinical manifestations of a glucagonoma

The Nekrolitichesky migrating erythema with secondary violent dermatitis (90%).
Disturbance of tolerance to glucose or a diabetes mellitus (90%).
Anemia, loss of body weight, anorexia.
Tromboembolic episodes with formation of venous blood clots (20%) and a pulmonary embolism (12%).
Metastasises in a liver and other abdominal organs.
Diagnosis
High level of a glucagon of blood (at 80% of patients exceeds norm by 5 — 10 times). Glucagon level more than 1000 pg/ml (the norm <200 pg/ml) is considered pathognomonic for a glucagonoma.
Hypoaminoacidemia (80%).
Topical diagnosis of a tumor and its metastasises.

Differential diagnosis

Diabetes mellitus, skin changes of other etiology, metastasises in a liver of other tumor.
Glucagon level in blood can be increased (usually moderately) at a chronic renal and liver failure, acute pancreatitis, sepsis, an acromegalia, a syndrome of Kushinga, long starvation.

Treatment

At the time of diagnosis developed a glucagonoma syndrome a radical operative measure, as a rule, it is already impossible. For anti-proliferative therapy and suppression of products of a glucagon it is most effective oktreotid (it is long the operating analog of somatostatin).

Forecast

In most cases adverse.

 
"Glomusangioma   Granuloma of bones eosinophilic"