Zollinger-Ellison syndrome Symptoms Diagnosis And Treatment
Zollinger Ellison syndrome (ZES) is a tumor of the endocrine pancreas that secretes gastrin and determines multiple ulcers that do not respond to treatment. SEZ is quite rare, statistics show that the incidence of this disease would be around 3/1000 000 inhabitants but it must be said that the disease may be undiagnosed in many cases. This endocrine tumor is found most often in people aged between 60 and 80 years. In 25% of cases it may be associated with MEN I syndrome, multiple endocrine neoplasia type 1, which is an autosomal dominant syndrome, and involves association of multiple tumors (pituitary tumors, parathyroid, pancreas tumors).
Most tumors are located in the pancreas, others are located in the stomach, duodenum, spleen, liver, lymph nodes, small intestine and ovary. In addition to gastrin, these endocrine tumors may release other hormones, such as insulin, glucagon, somatostatin, but in much smaller amounts. Gastrin is a hormone that acts on parietal cells in the stomach and stimulates them to produce hydrochloric acid. Hydrochloric acid is harmful to gastric mucosa and causes gastric ulcers.
Symptoms and signs
One of the hallmarks of ZES is the presence of multiple gastric and duodenal ulcers. ZES causes ulcers in almost all patients, but there can also occur other symptoms such as diarrhea, vomiting and weight loss. However it can happen that a patient has diarrhea without having ulcers, which occurs in approximately 30% of patients. Ulcer patients often have complications such as bleeding or perforation,which are medical emergencies. Another sign of the disease is esophagitis, that is damage to the esophagus from stomach acid.
About half of these endocrine tumors can become malignant and tend to disseminate to the liver. ZES often gives slow-growing tumors but sometimes it may happen that these tumors grow faster and cause early metastasis.
Diagnosis is suspected from clinical signs and confirmed by a number of biological and imaging investigations. Biological investigations show:
-Fasting serum-gastrin- over 100 pg / ml
-Gastric acid level over 30 mEq / l per hour
-Secretin stimulation test over 2 micrograms
Imaging investigations that can be used are:
– Abdominal ultrasound, which can detect the primary tumor in 20% of patients
-Computed tomography, PET-CT
– Octreotide scintigraphy
SEZ benefits of medical and surgical treatment. Medical treatment consists of proton pump inhibitors, which are the treatment of choice in patients with ulcer: omeprazole, pantoprazole. It can also be used H2 receptor inhibitors, such as ranitidine or famotidine. Although these drugs are intended to decrease gastric acid secretion, they are sometimes ineffective. Most effective pharmacological treatment seems to be octreotide.
Surgery aims to remove the tumor which secrete gastrin: pancreatic resection or gastrectomy may help.