Zollinger-Ellison syndrome is inherently a neoplasm of the duodenum or pancreas of the hormone-active type, producing gastrin and becoming the cause of peptic and duodenal ulcers.
This article will talk about the causes, some complications, manifestations, prognoses, diagnostic methods and treatment of the disease. This information will help to see the manifestation of the first signs of a dangerous pathology, which are very similar to the symptoms of peptic ulcer, and the patient will make the right decision about whether treatment is needed by a doctor. Symptoms and treatment of Zollinger-Ellison syndrome are presented below.
Statistics
In 85-90 percent of cases, the neoplasm begins to grow from the cells of the tail or head of the pancreas, in about 10-15 percent the tumor is located in the periphencreatic lymph nodes or in the lower parts of the duodenum. The formation of gastrin in the liver, spleen, or stomach is very rare. Such tumors grow most often slowly, but somewhere in 60-90 percent they are malignant. Metastases can invade the spleen, mediastinum, liver, and regional lymph nodes. Gastrinomas can have sizes up to 0.2-2 centimeters, in more rare cases - from five centimeters and above.
Not many people know what Zollinger-Ellison syndrome is (pictured).
How often does it occur?
This pathology is quite rare - in one or four patients from one million people during the year. Doctors note that at first this disease often disguises itself as a typical ulcerative pathology, and in 90-95% of patients find numerous peptic ulcers, the formation of which is caused by excessive production of hydrochloric acid. This disease can develop at any age (mainly in patients from twenty to twenty-five years) and is most often diagnosed in the stronger sex.
Causes
Scientists have not yet been able to determine the true causes that cause the appearance of Zollinger-Ellison syndrome.
- The main cause of the disease is caused by the uncontrolled constant release of gastrin in large volumes, which is produced by the neoplasm that has appeared in the body.
- About 25% of people with a similar disease have endocrine multiple adenomatosis of the first type, accompanied by inflammation of the tissues of both the pancreas and other glands (adrenal gland, pituitary, parathyroid and thyroid).
- In some patients, the syndrome develops in connection with hyperplasia of G-cells producing gastrin and located in the antrum section of the stomach.
- In addition, there are suggestions about the genetic origin of this pathology, when the disease is transmitted to the child from the mother.
Symptoms
The patient with Zollinger-Ellison syndrome has symptoms that are characteristic of a duodenal ulcer or stomach. The pains that arise in this case can differ in an atypical arrangement, are persistent in nature and often poorly treated according to the antiulcer scheme.
Patients most often complain of intense pain in the upper abdomen that appears on an empty stomach or after eating (as with peptic ulcer disease). When analyzing the mucous membranes of these sections of the digestive system, ulcers of various depths are revealed. In addition, such inflammations can cause perforation of the walls of the stomach or duodenum and the development of gastrointestinal bleeding.
About 35-60 percent of patients suffer from diarrhea, and in some people this manifestation is the only symptom of Zollinger-Ellison syndrome. At the same time, the chair is watery, half-decorated, contains many undigested pieces of food and fat.
The combination of diarrhea and pain is observed in most patients. In addition, people with Zollinger-Ellison syndrome have complaints such as:
- belching with a sour taste;
- heartburn and burning sensation behind the sternum;
- nausea;
- vomiting
- weight loss due to prolonged diarrhea.
What other symptoms of Zollinger-Ellison syndrome are there? About thirteen percent of patients develop severe esophagitis. In some patients, further esophageal strictures and peptic ulcers may appear.
Gastrinoma, which is diagnosed with instrumental manipulations, looks like a nodular dark red formation of a round shape with a dense consistency. It can be either single or multiple.
A patient with malignancy of gastrinoma develops symptoms of malignant intoxication, and cachexia is formed. With the appearance of metastases due to the tumor, signs of inflammation of the liver, mediastinum, lymph nodes and spleen develop.
Symptoms of Zollinger-Ellison syndrome are important to recognize in a timely manner.
Alleged Complications
The syndrome is able to have the following pathological conditions as complications:
- ulcerative perforation and the appearance of peritonitis;
- intestinal and stomach bleeding;
- strictures of the lower esophagus;
- defects in the activity of the heart that arise due to prolonged diarrhea, leading to a large loss of potassium;
- weight loss (maybe before cachexia);
- compression of the bile duct by the tumor, which leads to jaundice and indigestion and stomach;
- gastrinoma metastasis and malignancy.
What is the diagnosis of Zollinger-Ellison syndrome?
Diagnostics
With the early diagnosis of this syndrome, it is rather difficult to establish it, since the clinical manifestations are similar to an ulcer. In this case, the implementation of the following diagnostic measures is very important:
- Analysis of gastric juice with functional tests (pharmacological or nutritional with calcium salts and secretin).
- A blood test to determine the level of gastrin in serum. With Zollinger-Ellison syndrome in a patient, the analysis determines an increase in gastrin content to indicators of 1000 pg per ml or higher, as well as an increase in the level of hydrochloric free acid from four to ten times.
In addition to laboratory research data, analyzes such as:
- identification of the degree of chromogranin A;
- hormonal analysis (growth hormone, prolactin, insulin, etc.).
To assess the nature of inflammation of the internal organs, it is necessary to conduct the following studies:
- FGDS;
- CT
- Gastric radiography;
- MRI
- Ultrasound of the organs of the peritoneal cavity;
- ayudominal selective angiography, which measures the degree of gastrin in the veins of the pancreas;
- radioisotope bone scan;
- scintigraphy with radioisotopes labeled with somatostatin analogues.
If there is a suspicion of a disease such as Zollinger-Ellison syndrome, then differential diagnosis with other pathologies is necessary:
- long-term healing and frequently occurring ulcers in the digestive upper parts;
- neoplasms in the small intestine;
- hypergastrinemia due to gastritis, hyperthyroidism, B12-deficient anemia or pyloric stenosis;
- celiac disease.
Treatment for Zollinger-Ellison Syndrome
If this syndrome was diagnosed, then the patient is hospitalized in the surgical or gastroenterological department. With the malignant nature of the tumor, the patient is preferably treated in an oncological medical institution.
Conservative treatment
Drug therapy for Zollinger-Ellison syndrome has as its goal the reduction of acidity, as well as the prevention of new ulcerations, as well as the healing of existing ulcers in patients. For this patient, the following groups of drugs can be prescribed:
- H2-histamine blockers - “Ranitidine”, “Famotidine”;
- proton pump inhibitors - Omeprazole, Lansoprazole, Rabeprazole, etc .;
- m-anticholinergics - platifillin hydrochloride, "Pirenzepine";
- "Octreotide" (similar to "Stomatostatin").
To slow down the growth of a tumor or reduce its size, the doctor may prescribe cytostatics: Streptozocin, Doxirubicin, 5-fluorouracil, etc. Recommendations for Zollinger-Ellison syndrome should be strictly followed.
Surgery
If gastrinoma metastases are absent, then in order to remove it, a type of minimally invasive surgery can be performed such as fiber optic diaphanoscopy of the duodenum and duodenotomy lateral with further revision of the mucosa. In more complex situations and when it is impossible to determine the exact location of the neoplasm, a total gastrectomy or gastric resection with pyloroplasty or proximal vagotomy is performed.
Complications
Due to the ignoring of symptoms and inadequate treatment, various complications can occur. Thus, the consequences of gastrinoma are as follows:
- perforation of a duodenal ulcer or stomach, peritonitis;
- the lumen of the esophagus narrows;
- there are hemorrhages in the gastrointestinal tract, as a result anemia occurs;
- body weight decreases, an extreme degree of exhaustion sets in;
- the tumor is transformed into a malignant, metastases occur;
- bile ducts are compressed by a neoplasm, which leads to jaundice;
- the work of the heart is disturbed.
We examined the symptoms, diagnosis, and treatment of Zollinger-Ellison syndrome.