Typical (TC) and atypical (AC) carcinoids are low-grade neuroendocrine tumors (NETs) of the lung and, are neglected diseases in respect of both high-grade NETs of the lung (i.e. small-cell lung cancer and large-cell neuroendocrine carcinoma) and gastroenteropancreatic (GEP)-NETs. AC and TC account for 2 and 0.2% of all thoracic malignancies, respectively, and have a 12.9% chance of metastatic spread at diagnosis, reaching up to 20% during disease history. There are very few trials specifically designed for lung NETs, and therapeutic options are mainly derived by studies carried out in patients with GEP-NETs. We report a case of a patient affected by AC progressed to available standard treatments who received off-label treatment with sunitinib, a well-known multitarget tyrosine-kinase inhibitor with marked antiangiogenic activity, used routinely for the treatment of GEP-NETs. During treatment, the patient required the administration of an alternative schedule to improve tolerability, with benefit, and achieved a partial response according to the RECIST criteria, which is unusual in NETs. We critically reviewed available data supporting the use of somatostatin analogs, chemotherapy, and target therapies (everolimus and sunitinib) in advanced lung NETs. In the review, ongoing trials in lung NETs and future developments in this research field are also discussed.
aNET Team Bologna – ENETS Center of Excellence
bDepartment of Specialized, Experimental and Diagnostic Medicine
cDepartment of Internal Medicine and Surgery, S. Orsola-Malpighi Hospital
d’Giorgio Prodi’ Cancer Research Center, Alma Mater Studiorum, University of Bologna, Bologna, Italy
Received 23 January 2019 Revised form accepted 28 February 2019
Correspondence to Giuseppe Lamberti, MD, Department of Specialized, Experimental and Diagnostic Medicine, Alma Mater Studiorum, University of Bologna, S. Orsola-Malpighi Hospital Via Massarenti 9, 40138 Bologna, Italy Tel: + 39 051 636 3067; fax: + 39 051 636 4136; e-mail: firstname.lastname@example.org