Zollingera-Ellison's syndrome

Description:

Zollingera-Ellison's syndrome (pancreas adenoma ulcerogenic, a gastrinoma) — the tumor of the insular device of a pancreas which is characterized by emergence of the round ulcers of a duodenum and stomach which are not giving in to treatment and followed by persistent ponosa.

Symptoms of the Syndrome of Zollingera-Ellison:

Clinical displays of a disease are pains in an upper part of a stomach which have the same patterns in relation to meal, as at a usual ulcer of a duodenum and stomach, but unlike them are very persistent, differ in big intensity and do not give in to antiulcerous therapy. Persistent heartburn and an eructation acid are characteristic. An important sign are the ponosa caused by hit in a small intestine of a large amount of hydrochloric acid and strengthening thereof motility of a small bowel and absorption delay. The chair is plentiful, watery, with a large amount of fat. Perhaps considerable decrease in body weight that is characteristic of a malignant gastrinemiya.
Stomach ulcer and duodenum at Zollinger's syndrome — Ellisona do not give in to healing even at long corresponding therapy. At many patients the esophagitis phenomena, sometimes even with formation of an esophageal stenosis are observed. Palpatorno is defined the expressed morbidity in an upper part of a stomach, and area of a projection of the lower part of a stomach, there can be a positive symptom of Mendel (local morbidity in an ulcer projection). In case of malignant disease tumoral educations in a liver and its significant increase are possible.
X-ray and endoscopic inspection reveal an ulcer which does not differ from an ulcer at a usual peptic ulcer of a duodenum.
Relative laboratory criteria of a syndrome of Zollinger — Ellisona are:
    * a gipergastrinemiya (content of gastrin in blood to 1000 pg/ml and more, at a usual peptic ulcer it does not exceed the upper bound of norm — 100 pg/ml);
    * acidity of the main secret more than 100 mmol/h.
Visualization of a tumor is made by means of ultrasonography, a computer tomography, the selection abdominal angiography. The greatest informational content the method of the selection abdominal angiography with capture of blood from pancreatic veins and definition of gastrin in it has.

Reasons of the Syndrome of Zollingera-Ellison:

The syndrome results from the tumor which is located in the field of a head or a tail of a pancreas (85% of cases). In 15% of cases the tumor is localized in a stomach or is display of multiple endocrine adenomatosis (a multiple endocrine neoplasia). Development of the round ulcers steady against treatment is connected with the increased development of a gastric juice and, respectively, hydrochloric acid and enzymes.
At the vast majority of patients it is localized in a duodenum, it is found in a stomach and a jejunum less often. Multiple stomach ulcers, duodenal and a jejunum are often observed.

Treatment of the Syndrome of Zollingera-Ellison:

At most of patients with Zollingera-Ellison's syndrome usual conservative and surgical methods of treatment of a peptic ulcer are inefficient. Quite often after a stomach resection (with vagisection or without it) or vagisections with an ulcer pyloroplasty very quickly recur.
In the past many patients from gastrinomy were exposed to multiple operations, especially when from the very beginning it was not possible to establish the diagnosis. Mortality at patients to whom carried out a gastrectomy at once was lowest. Therefore the gastrectomy long time was considered as a choice method at treatment of a gastrinoma.
Creation of the powerful medicines suppressing secretion of hydrochloric acid and development of exact methods of topical diagnosis by gastrin significantly expanded possibilities of treatment.
Clinical manifestations and disease severity are various therefore approach has to be individual. As well as at other malignant new growths, an ideal method of treatment is the oncotomy.
H2 blockers reduce secretion of hydrochloric acid, facilitate symptoms and promote healing of ulcers. These drugs are effective at 80-85% of patients.
Reception of the same drugs is recommended, as at a peptic ulcer of a duodenum, but their doses have to be in 4-8 times more. H2 blockers are appointed for life as even at the temporary termination of their reception there is an ulcer recurrence. Approximately at 25% of patients of an ulcer will not respond to treatment of H2 blockers or recur during treatment. The dose of drugs is selected depending on the level of basal secretion of hydrochloric acid measured within an hour before the next administration of drug. This indicator should not exceed 2,8 µmol / with (10 мэкв / h). H+,K inhibitors +-Atfazy (омепразол and лансопразол) are most effective for suppression of secretion of hydrochloric acid and treatment of ulcers at Zollingera-Ellison's syndrome, including at resistance to H2 blockers. The initial dose of an omeprazol and a lansoprazol makes 60 mg once in the morning till a breakfast. The dose is selected so that basal secretion of hydrochloric acid did not exceed 2,8 µmol / with (10 мэкв / h), and at the operated patients - 1,4mkmol/with (5 мэкв / h). At an esophagitis decrease in secretion to 0,25 µmol / with can be required (1 мэкв / h). A third of patients needs to accept drug 2 times a day. Sometimes after improvement of a state it is possible to lower its dose. When it is not possible to reveal a tumor or to remove it, sometimes resort to proximal selection vagisection. At some patients it allows to lower a dose of H2 blockers and even completely to repeal them.
At the choice of a method of treatment it is necessary to take clinical displays of a disease into account. Earlier such patients perished generally from complications of a peptic ulcer. However improvement of early diagnosis and successful antiulcerous therapy led to increase in life expectancy of patients because of what malignant properties by gastrin even more often began to be shown. About a half of patients which did not remove a gastrinoma die of germination by a tumor of surrounding fabrics and the next bodies. An optimum method of treatment - a full oncotomy. During operation it is necessary to carry out a fiber-optical diafanoskopiya of a duodenum and a lateral duodenotomy with careful survey mucous. Advantages of intraoperative ultrasonography should be found out. The full removal of a gastrinoma leading to treatment is possible only at 30% of patients. Before establishment of the diagnosis, specification of localization and the sizes of a tumor and carrying out operation appoint омепразол or лансопразол. In the presence of contraindications, not operability of a tumor or in case of refusal the patient from operation should accept these drugs for life. For reduction of tumoral weight and the accompanying symptoms at the gastrinoma inclined to invasive growth, tried to apply a combination of a streptozotsin, a ftoruratsil and doxorubicine. Remission after chemotherapy is observed less than at 65% of patients and never happens full. Efficiency of interferon an alpha and an oktreotida is also small. At an inoperable gastrinoma or existence of metastasises for prevention of formation of ulcers appoint омепразол or лансопразол. Carry out a gastrectomy less often. Influence of a gastrectomy on the speed of a tumoral progression is not proved.