Onkológia 5/2017

Treatment of pulmonary neuroendocrine tumors

Neuroendocrine lung tumors (pNET) characterize heterogeneous and inconsistent biological behaviors within a group. We rank them among rare malignancies and in all neuroendocrine tumors they represent approximatly 60 %.. One reason for their heterogeneity is a different grading. The basis for the treatment of chemosensitive lung carcinoids is surgical resection, the cornerstone for the treatment of small cell lung carcinoma has for many years been a combination of chemotherapy and radiotherapy. Large-cell neuroendocrine pulmonary carcinoma is less chemosensitive and responses to irradiation are smaller than for small cell lung carcinoma, the most appropriate procedure is surgical resection of early stages with adjuvant chemotherapy. On the opposite side of the spectrum, there are also prognostic indicators of survival of well and poor differentiated lung neuroendocrine tumors. Significant progress has been made in the description of signaling pathways in carcinoid cells, and the most prudent PI3K / Alt / mTOR cascade appears to be. The approval of everolimus in February 2016 for the progression of a well differentiated, non-functional, initially unreseable pulmonary carcinoid is a significant advance in medical treatment. In the case of poor differentiated pulmonary neuroendocrine tumors, such a breakthrough study is lacking, and prospective research into targeted treatment and immunotherapy appears. Promise to future are currently ongoing studies with pembrolizumab (PD-1 inhibitor), and rovalpituzumab tesiren , an antibody-drug conjugate targeted to DLL3 (delta like protein 3) on the surface of tumor. DLL3 is expressed in approximately 70 % of patientswith small cell lung cancer.

Keywords: neuroendocrine lung tumors, pulmonary carcinoid, small cell lung carcinoma, large cell neuroedocrine lung carcinoma