Abstract

Introduction: Gastroenteropancreatic neuroendocrine tumors (GEP-NET) are classified on the basis of hormonal activity of tumor cells to functional and non-functional tumors. Therapy of well differentiated NETs includes surgical procedures, debulking of tumor mass, biotherapy and peptide receptor radionuclide therapy. Multidisciplinary approach in diagnostic and therapy leads to prolongation of survival. Aim of the study: Analysis of therapeutic modalities in group of patients with well differentiated gastroenteropatic neuroendocrine tumors and influence of tailored therapy for prognosis of patients. Characteristics of patients: In our department we followed up in period from 1.1.2007 to 31.3.2013 65 pts (21 men/44 women) with well differentiated GEP neuroendocrine tumors. Primary localisation was: stomach — 6 times, pancreas — 9×, duodenum — 1×, jejunum — 4×, appendix — 8×, ileum — 26×, rectum — 7×, unknown primary — 4×. On the base of histomorphological diagnosis 47 patients belonged to WHO I. group — very well differentiated neuroendocrine tumors, and 18 pts to WHO II. group — well differentiated neuroendocrine carcinomas. Metastatic disease was affirmed in 36 patients in time of diagnosis. In all patients chromogranin A, urine level of 5-HIAA, hormonal analysis were examined. Besides ultrasound, endoscopy, computer tomography also Octreoscan was performed. Carcinoid syndrome was present in 27 patients: in 23 pts primary tumors were localised in midgut, in 4 pts we did not find the primary tumor. In our group 9 patients had neuroendocrine tumor of pancreas, 4 of them were functional: 2× overproduction of calcitonine, 1× of gastrin,1× of insulin. Therapy: A. Surgical treatment: Resection of primary tumor and debulking of metastases was performed in 56 pts, in 5 pts with pancreatic tumor resection was not possible due to invasion of tumor mass to surrounding tissue and vessels. In 4 patients with unknown primary surgical treatment was not performed. B. Biological treatment with long acting somatostatin analogues was indicated in 37 pts, 4 pts are treated also with m-tor inhibitors. Biotherapy with somatostatin analogues reduced symptoms of hormonal activity (decrease of 5-HIAA in urine) and brought on stabilisation of disease in most of patients. C. Peptide receptor radionuclide therapy (PRRT) was indicated in 8 pts, 4 of them with nonresectable neuroendocrine carcinoma of pancreas. In 6 of patients PRRT with 90 Ytrium–DOTA-octreotid and in 2 patients with 123 I-MIBG. Reduction of tumor volume was noticed. In period of follow up 8 patients died. Reasons of death: in 2 pts right side heart failure due to fibrosis of endocardium, in 1 patient — small bowel necrosis due to desmoplastic reaction of mesenterium, in 4 patient — malignant cachexia, in 1 patient — carcinoid crisis. Conclusion: Patients with GEP-NETs demand on multidisciplinary approach in therapy. Surgical procedure, biological therapy with somatostatin analogues, m-tor inhibitors and PRRT in patients with well differentiated neuroendocrine tumors markedly contribute to prolongation of survival of patients and also to enhancement quality of their life. We supposed that early combination treatment has high influence for best survival of patients with metastatic disease.

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