Glucagonoma
Description:
The glucagonoma is the quite rare tumor growing from and - cells of islands of a pancreas. The tumor produces a glucagon and is followed by very characteristic rashes. They are presented by the nekrolitichesky migrating erythema, a glossitis and a perleche. The Nekrolitichesky migrating erythema received the name because of the tendency to peripheral growth; erosion, crusts and a postinflammatory hyperpegmentation are characteristic of it.
Glucagonoma reasons:
Glucagonomas are found in people of middle and advanced age more often.
The majority of cases of the nekrolitichesky migrating erythema are caused by surplus of a glucagon in blood, however its pathogeny is unknown.
Glucagonoma symptoms:
On the patient's skin with a glucagonoma the rashes which are not giving in to treatment are observed. Weight loss, abdominal pains takes place.
Rash elements. The migrating nekrolitichesky erythema: the inflamed plaques with the regional growth and healing in the center. Plaques merge that gives to the defeat centers a type of the map. At the edges of plaques bubbles, crusts and scales are formed.
Arrangement. Spiral-shaped, ring-shaped, the arc-shaped.
Localization. Flexion surfaces of extremities, large skin folds, circle of a mouth, external genitals and anus. Finger-tips of hands red, brilliant, with erosion.
Mucous membranes.
The blepharitis, glossitis (big fleshy red language with smoothed nipples), a perleche develops.
The migrating nekrolitichesky erythema at the patient from glkogonomy
Diagnosis:
Biochemical analysis of blood.
The diagnosis of a glucagonoma is made at plasma glucagon level on an empty stomach more than 1000 ¡ú/l (norm — 50 — 250 ¡ú/l). So high concentration of a glucagon causes disturbance of tolerance to glucose and a hyperglycemia. Heavy disturbances of absorption, sharp decrease in level of amino acids and decrease in level of zinc in serum are characteristic.
Patomorfologiya of skin.
At early stages — a polosovidny necrosis of upper layers of epidermis, a keratinotsita with pale cytoplasm, pycnosis of kernels. At a submicroscopy gidropi-chesky dystrophy and a lysis of organellas are visible.
KT, angiography.
Allow to establish localization of a tumor.
Tissue specimen of a tumor of a glucagonoma
Treatment of a glucagonoma:
The main manifestation of a glucagonoma - the nekrolitichesky migrating erythema - will very badly respond to treatment. At a number of patients replacement therapy by zinc is effective.
treatment.
Because of the high frequency of innidiation (most often in a liver) removal of a glucagonoma leads to recovery in only 30% of cases. However decrease in mass of a tumor is followed by involution or even total disappearance of symptoms of a disease, including the nekrolitichesky migrating erythema.
Chemotherapy.
It is usually ineffective.
