Neuroendocrine tumor G3 of bronchopulmonary origin and its classification

Neuroendocrine tumors (NET) with high proliferative activity (Ki-67 index >20% and/or mitotic counts >2 mm(2)) are defined as NET G3 in the 2019 World Health Organization (WHO) classification of digestive system neuroendocrine neoplasms (NENs). NETs G3 occur mostly in the pancreas, colon, rectum, and stomach and only rarely in the small intestine and the appendix. In the bronchopulmonary system, similar tumors have also been recognized and were mostly classified as atypical carcinoid (AC) or large cell neuroendocrine carcinoma. Bronchopulmonary NENs that were classified as NETs G3 are characterized by histological and immunohistochemical similarities with carcinoids/NETs, and a clinical course that is more aggressive than with ACs and similar to that of neuroendocrine carcinomas. The morphomolecular and clinical features of bronchopulmonary neoplasms with a high proliferative activity were reviewed and a future classification system that is applicable for both digestive and bronchopulmonary NETs is proposed.

Automated summary

Neuroendocrine tumors with high proliferative activity are classified as NET G3 in the 2019 WHO classification. They occur mainly in the pancreas, colon, rectum, and stomach, and only rarely in the small intestine and appendix. Similar tumors have been found in the bronchopulmonary system and are classified as atypical carcinoid or large cell neuroendocrine carcinoma. A future classification system that applies to both digestive and bronchopulmonary NETs is proposed.