Neuroendocrine tumors of the lung: a comprehensive overview

A total of 25% of neuroendocrine tumors originate in the lung. Still, typical and atypical carcinoids of the lung are rare, accounting for only 1-2% of all lung cancers. Distant metastases are infrequent, occurring in 2-14% of cases. The mainstay of treatment of local disease is surgery. In advanced disease there are no antiproliferative agents approved for carcinoids of the lung. None of the currently available drugs provide a cure. There are several treatment options, such as somatostatin analogs, mTOR inhibition, inhibitors of angiogenesis, systemic chemotherapy and radiolabeled somatostatin analogs. Interpretation of the data is complicated, since it mainly consists of small (retrospective) Phase II studies. Fortunately, randomized Phase II and III studies are underway. This article emphasizes the specific features of neuroendocrine tumors in the lung and focuses on the treatment in advanced disease.