RESUMO
Neuroendocrine tumors (NETs, originally termed "carcinoids") create a relatively rare group of neoplasms with an approximate incidence rate of 2.5 to 5 cases per 100 000 persons. Roughly 30% to 40% of subjects with NETs develop carcinoid syndrome (CS), and 20% to 50% of subjects with CS are diagnosed with carcinoid heart disease (CaHD). The long-standing exposure to high serum serotonin concentration is one of the crucial factors in CaHD development. White plaque-like deposits on the endocardial surface of heart structures with valve leaflets and subvalvular apparatus thickening (fused and shortened chordae; thickened papillary muscles) are characteristic for CaHD. NT pro-BNP and 5-hydroxyindoleacetic acid are the 2 most useful screening markers. Long-acting somatostatin analogs are the standard of care in symptoms control. They are also the first-line treatment for tumor control in subjects with a metastatic somatostatin receptor avid disease. In cases refractory to somatostatin analogs, several options are available. We can increase a somatostatin analog to off-label doses, add telotristat ethyl or administer peptide receptor radionuclide therapy. Cardiac surgery, which mainly involves valve replacement, is presently the most efficient strategy in subjects with advanced CaHD and can relieve unmanageable symptoms or be partly responsible for better prognosis.
RESUMO
Neuroendocrine neoplasms (NEN) are group of rare neoplasms, which frequency is estimated on about 35 cases/100000. Though, during last 30 years the number of NEN new cases increased five-times. Nowadays the only method which allows to cure NEN is surgical treatment. Laparoscopic surgery and endoscopic treatment are also used. These neoplasms are usually diagnosed in advanced stadium, with distant metastases, when surgery is not an option. In this group of patients systemic and local therapies are used, such as somatostatin analogues, chemotherapy or targeted therapy. The choice of proper method is determined not only by neoplasm's localization or its size, but also clinical symptoms caused by tumor itself or by substations released by it. One place hopes in novel molecular-based therapies and currently investigated therapies using i.a. oncolytic viruses.
