Endocrine pancreas cancer-Somatostatinomul

Endocrine pancreas cancer-Somatostatinomul

    * Introduction
    * Signs and symptoms
    * Diagnosis
    * Treatment
Somatostatinomul is a tumor derived from D cells that secrete somatostatin pancratice islands. Syndrome causes diarrhea, steatorrhea, weight loss, hypochlorhydria, diabetes and coledocolitiaza. It is usually located distal pancreatic or corporeal but can be found in the duodenum. Tumors are larger than 3 cm in diameter. Most are malignant metastasized frequently.
In 7% of cases somatostatinomul syndrome is associated with multiple endocrine neoplasia MEN I type, involving: parathyroid neoplasms, pituitary and pancreas. Neurofibromatosis and pheochromocytoma are associated with the duodenal somatostatinomului form. Diagnosis is made by measuring serum levels of somatostatin and computed tomography techniques to locate and staged tumors. Somatostatinoamele evolve undetected for years so are very high, over 5 cm at diagnosis. 80% of patients have metastases at diagnosis and 50% of those with duodenal tumors had evidence of liver metastasis, lymph nodes or bone. 40% of patients died at intervals ranging between one week and 14 months after diagnosis, while 60% supravietuies from 6 months to 5 years.
Surgical resection is the only option that can cure the disease. The aims of surgery are due to excess hormonal symptoms controlulo, rezecarea efficient tumor and healthy pancreatic parenchyma preservation. Chemotherapy is used in tumor dissemination and consist of a combination of 5-fluorouracil and intravenous streptozotocin. 50% of patients had a favorable response to this therapy. Patients with unresectable and metastatic somatostatinoame symptomatic require specific treatment. Diabetes can be controlled associated with oral agents and less insulin is needed. Pancreatic enzymes are useful in reducing diarrhea and steatorrhea.
Pathogenesis and causes
Somatostatin is a peptide that acts to inhibit endocrine and paracrine secretion of certain hormones, among which insulin, glucagon, growth hormone, gastrin, cholecystokinin, secretin and VIP. Lowers the inhibitory action of gall bladder contractility, decreased exocrine pancreatic function, intestinal secretion and motility.
Reduced insulin secretion lowers peripheral glucose utilization and hepatic overproduction waging diabetes. Clinical severity may vary from state to diabetic glucose intolerance prior to frank ketoacidosis. Coledocolitiaza and biliary tract disease develops in 25-68% of patients secondary colecistokinazei suppression, alteration of bladder and moilitatii fat metabolism.
Somatostatinomul often occur simultaneously and other hormones: insulin, gastrin, VIP, glucagon, corticotropin, calcitonin, pancreatic polypeptide.