RESUMEN
UNLABELLED: The endocrine part of the pancreas consist 2% from the mass of the gland and it is built from little cells with name Langerhan's islands. The increased secretion of cells provokes to appearance of classic clinical syndrome, which is conditioned by large quantity of hormone in circulation of the blood. The endocrine tumors of the pancreas (ETP) are arisen out of cells that have large quantity to APUD-system. MATERIAL AND METHOD: We present a case report of rare ETP with secretion of cortisol (MEN2 syndrome), which is of interest by reason of the fact that this case report is the first announce in Bulgaria about similar tumor. Corporocaudal resention of the pancreas with spleenectomy has been made. RESULTS: Tumor cells with highly expresion (+++) of hromogranin were determined. The histological conclusion was malignant ETP (APUD system tumor)with secretion of cortisol. A decrease of the level of glucose in blood circulation, a decrease of blood pressure and reduction of the body weight were determined with range of first three postoperative weeks. CONCLUSION: The aims of the surgical therapy are a control over the level of hormones, clear resection and maximal organ saving operation.