Lung well-to-moderately differentiated neuroendocrine tumors (also known
as carcinoids) and large cell neuroendocrine lung carcinoma (poorly
differentiated neuroendocrine tumor) are rare neuroendocrine neoplasms,
which account for less than 4% of all lung neoplasms. Due to their low
incidence, their systemic treatment is greatly influenced by therapeutic
evidence derived from the more frequent gastroenteropancreatic
neuroendocrine neoplasms and/or small cell lung carcinoma leading to
significant bias. Currently, employed systemic therapies for lung
carcinoids, aiming at controlling tumor growth include long acting
somatostatin analogues (SSAs), peptide receptor radionuclide therapy,
chemotherapy and molecular-targeted therapy. In this review, each of
those treatments is presented based upon available clinical evidence
from retrospective and prospective studies particularly focused on the
role of everolimus in the advanced setting and on ongoing clinical
trials reflecting our expectations in the near future. In addition, we
critically analyse currently employed treatment of large cell
neuroendocrine carcinoma where the appropriate chemotherapeutic regimen
is still a matter of debate