The author aims to review the established medical treatment options of neuroendocrine tumours, which have expanded greatly in recent years and present the most important aspects to be considered in planning patients' management. Medical treatment is usually considered in advanced stages of these tumours, as well as in cases of
hormone overproduction.
Somatostatin analogues have been known to be effective in alleviating
hormone excess syndromes, especially
carcinoid syndrome for the past 25 years. There is a convincing evidence that the
somatostatin analogue
octreotide is useful as an
antitumor agent, at least in well-differentiated small intestinal neuroendocrine tumours and probably also in those of pancreatic origin.
Interferons may be also used and the indications for their use may be almost the same. Optimal patient selection is mandatory for the use of cytotoxic
chemotherapy.
Streptozotocin- and, recently,
temozolomide-based
chemotherapies should be considered in progressive phases of well differentiated (G1/G2) pancreatic neuroendocrine tumours. A
cisplatin-
etoposide combination is the first choice for the treatment of G3
neuroendocrine carcinomas of any origin. Recently, the
mammalian target of rapamycin inhibitor
everolimus and the combined
tyrosine kinase inhibitor sunitinib were registered for the treatment of G1/G2 pancreatic neuroendocrine tumours. The most recent
drug treatment recommendations and therapeutic algorithms to improve systemic
therapy in patients with neuroendocrine tumours are summarized and novel
drug candidates with particular potential for future management of these tumours are outlined.