Table 1 NEN 2018 WHO proposed classification of selected NEN by site, category, family, and tumor type

From: A common classification framework for neuroendocrine neoplasms: an International Agency for Research on Cancer (IARC) and World Health Organization (WHO) expert consensus proposal

Site

Category

Family

Type

Grade

Current terminology

Lung

Neuroendocrine neoplasm (NEN)

Neuroendocrine tumor (NET)

Pulmonary neuroendocrine tumor (NET)a

G1

G2

Carcinoid

Atypical carcinoida

Neuroendocrine carcinoma (NEC)

Small cell lung carcinoma

(Pulmonary NEC, small cell-type)b

 

Small cell lung carcinoma

Pulmonary NEC, large cell-type

 

Large cell NE carcinoma

Uterus (corpus and cervix)

Neuroendocrine neoplasm (NEN)

Neuroendocrine tumor (NET)

Uterine neuroendocrine tumor (NET)

G1

G2

G3

Carcinoid

Atypical carcinoid

Atypical carcinoid

Neuroendocrine carcinoma (NEC)

Uterine NEC, small cell-type

 

Small cell carcinoma

Uterine NEC, large cell-type

 

Large cell NE carcinoma

Pancreas

Neuroendocrine neoplasm (NEN)

Neuroendocrine tumor (NET)

Pancreatic neuroendocrine tumor (NET)

G1

G2

G3

PanNET G1

PanNET G2

PanNET G3

Neuroendocrine carcinoma (NEC)

Pancreatic NEC, small cell-type

 

Small cell NE carcinoma

Pancreatic NEC, large cell-type

 

Large cell NE carcinoma

  1. NEC are regarded as high grade, but as they represent a separate tumor family, there is no need to for formal grading.
  2. aThe category of G3 atypical carcinoid in the lung is not a validated entity and not recognized in the 2015 WHO classification. Currently such tumors are classified as small cell lung carcinoma (SCLC) or large cell neuroendocrine carcinoma (LCNEC). High-grade NET with features of atypical carcinoid similar to the G3 tumors of the pancreatic/gastrointestinal tract are rare in the lung, not well characterized and need further study.
  3. bNot recommended as small cell lung carcinoma (SCLC) is too well ingrained in clinical practice and some SCLC lack commonly used neuroendocrine markers.