NCI Definition: A low, intermediate, or high grade malignant neoplasm with neuroendocrine differentiation that arises from the lung. This category includes typical carcinoid tumor, atypical carcinoid tumor, small cell carcinoma, large cell neuroendocrine carcinoma, and combined carcinoma. [1]

Lung neuroendocrine neoplasms most frequently harbor alterations in TP53, RB1, KMT2D, NOTCH1, and CREBBP [2].

Most Commonly Altered Genes in Lung Neuroendocrine Neoplasm

TP53 Mutation, TP53 c.217-c.1178 Missense, TP53 Missense, RB1 Mutation, and KMT2D Mutation are the most common alterations in lung neuroendocrine neoplasm [2].

Top Alterations in Lung Neuroendocrine Neoplasm

Significant Genes in Lung Neuroendocrine Neoplasm

MLH1 +

MLH3 +

MSH2 +

MSH3 +

MSH6 +

PMS1 +

PMS2 +

Disease Details

Neuroendocrine Neoplasm of the Lung, Neuroendocrine Neoplasm of Lung, Pulmonary Neuroendocrine Neoplasm
Neuroendocrine Neoplasm
Lung Carcinoid Tumor, Small Cell Lung Carcinoma, Combined Large Cell Lung Neuroendocrine Carcinoma, and Large Cell Lung Neuroendocrine Carcinoma
OncoTree Name
Lung Neuroendocrine Tumor
OncoTree Code


1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 6. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.