Neuroendocrine neoplasms (NENs) are considered rare diseases because of their low incidence and comprise a heterogeneous group of tumors that span from well-differentiated low-grade relatively indolent tumors (NET) to poorly-differentiated high-grade aggressive ones (NEC). Nevertheless, NENs are highly prevalent in the general population and their incidence has been constantly rising in the last decades. Furthermore, NENs biological and clinical heterogeneity poses unique challenges in the management of patients with NEN.
Therapeutic options for these patients are limited, reflecting the poor insight that we yet have about the biology of these group of tumors, as well as their rarity, which makes research efforts challenging. The findings that NET commonly express somatostatin receptors and the key role of the mTOR pathway has prompted somatostatin analogues (with or without an associated radionuclide) and everolimus to be established therapeutic options in a wide array of NENs. However, further therapeutic options in patients with NET are critical to extend survival. When it comes to NECs, no shared standard is available and most of the therapeutic management is extrapolated from small-cell lung cancer (SCLC) treatment. Despite the newly discovered four molecular subtypes on SCLC, which has fostered new intriguing research scenarios in high-grade NEC, our understanding of this tumor is very limited. Improving the clinical and biological understanding of NENs is crucial to improve patients’ management and consequently their outcomes, as well as to identify new potential treatment strategies, which are a highly unmet need in these patients.
The aim of this Article Collection is to collect and highlight recent advances in the context of clinical management and biology understanding for NENs.
Dr. Lamberti is an Assistant Professor at the Università di Bologna (Italy) and clinical research fellow at Dana-Farber Cancer Institute (Boston, MA). His main research interests are neuroendocrine tumors, neuroendocrine and epithelial thoracic neoplasia, and methods in clinical cancer research, and he has authored more than 50 publications in peer-reviewed international scientific journals.
