Published online May 24, 2025. doi: 10.5306/wjco.v16.i5.106630
Revised: March 21, 2025
Accepted: April 1, 2025
Published online: May 24, 2025
Processing time: 77 Days and 3.7 Hours
Neuroendocrine neoplasms are a group of tumors with heterogenous malignancy that evolve from neuroendocrine cells, most frequently in the gastrointestinal tract and in the lung. The latest 2021 World Health Organization (WHO) classification of lung tumors defines neuroendocrine neoplasms of the lung as an independent group of tumors, including typical and atypical neuroendocrine tumors and small cell and large cell neuroendocrine carcinomas. Although the overall nomenclature is essentially unchanged from the fourth WHO classification, there are several clinically relevant updates. In this review article, we discuss the epidemiological, clinical, diagnostic, therapeutic and prognostic features of these fascinating neoplasms, including the latest insights, current challenges and future pers
Core Tip: Neuroendocrine neoplasms (NENs) arise from neuroendocrine cells and vary in malignancy, commonly affecting the gastrointestinal tract and lungs. The 2021 World Health Organization classification of lung tumors categorizes lung NENs into typical and atypical neuroendocrine tumors and high-grade small cell and large cell neuroendocrine carcinomas. While the classification remains largely unchanged, key clinical updates have been introduced. This review explores the epidemiology, diagnosis, treatment, and prognosis of lung NENs, highlighting emerging insights, and future directions.