Interní Med. 2007; 9(11): 514-519
Tumors of the neuroendocrine system
- 1 Endokrinologický ústav, Praha
- 2 Subkatedra endokrinologie IPVZ Praha
Neuroendocrinne system (NES) is a complex neuro-endocrine network of non well-defined origic (neural crest cells? pluripotent cells?) with varios function incl. paracrine and endocrine secretion and neuromodulatory function. Clinical importance of disorders of NES is not yet well established. From cells of NES arise neuroendocrine tumors (NETs), NETs secrete many hormonaly active substances that lead to various endocrine syndromes a NETs are malignant in nature, grade of malignity is usuelly low. There are localized predominantly in gastrointestinal system. Diagnosis can be estabilished by morphological methods, for clinical diagnosis estimation of hormonaly active substances only and for their metabolites widely used. Localization of NETs includes RTG methods (incl. CT). MR and scintigraphy with radiolabeled analog of somatostatin (octreoscan). From a broad spectrum of NETs most important is carcinoid, gastrinom, insulinom, very rare is vipom, glucagonom and somatostatinom. NETs also cause paraneoplastic syndromes incl. Cushing syndrome, Schwartz-Bartter´s syndrome and paraneoplastic hypercalcemia. Treatment NETs is medical and surgical. Even non-radical (paliative) surgery can significantly improve clinical status and probably prolange the life. Medical treatment includes application of somatostatin analogues and interferon. Antitumorous cytostatic treatment is not very effective as well as external irradiation. New therapeutic modalities are at the begining of clinical practice.
Keywords: neuroendocrine system, neuroendocrine tumors, carcinoid, gastrinom, insulinom, glucagonom, somatostatinom, vipom – definition, diagnosis, treatment
Published: January 1, 2008 Show citation
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